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Surgery Rotation (Sx, Heme, Musculoskeletal)

Terms in this set (205)

What is the most common embolic source of acute arterial occlusion in the lower extremities?

A Atrial fibrillation
B Aortic aneurysm
C Myocardial infarction
D Prosthetic cardiac valve
E Iliac artery thrombus

Atrial Fibrillation
The heart accounts for 80% of all emboli, with atrial fibrillation making up 70% of that. Aortic aneurysms are frequently lined with thrombus but infrequently embolize; aneurysmal disease only accounts for 6% of all acute arterial occlusion. Acute myocardial infarction (especially those associated with left ventricular thrombus) accounts for 25% of cardioembolism, with peripheral embolization often the first sign of a previously "silent" MI. Prosthetic cardiac valves make up a still small but increasingly prevalent source of emboli. Peripheral arterial thrombi account for only 3% of acute occlusion.

While covering the ED, you attend to a 12-year-old boy who cut his hand while trying to slice a bagel. His mother wrapped his hand with a dishrag and drove him to the ED. On exam, his vitals are stable; he has remained alert and cooperative but the rag has soaked through; his fingers feel numb. On removal of the rag, there is a fairly deep looking straight-edged, transverse laceration running the length of his palm, with overlying clotted blood that begins to actively bleed.

In overall wound management, what is the first priority in the evaluation in this patient?

Answer Choices
1 Debridement of any devitalized tissue
2 Thorough wound cleansing with removal of debris
3 Hemostasis
4 Thorough history of the wound mechanism and patient health including tetanus immunization history
5 Early closure of the wound

Hemostasis
Explanation
Foremost in the evaluation of a wound, in any setting, is hemostasis. Large amounts of blood pooling or notable pulsations warrant urgent exploration for arterial damage, which could need emergent clamping and ligation.

To provide healthy tissue for approximation during closure, debridement of devitalized tissue may be necessary if there is obvious necrosis.

Wound cleansing and debridement are most important in the prevention of secondary infections. Cleansing is best performed with normal saline under mild pressure using a 14-gauge angiocatheter on a 30-60 cc syringe. This may require use of local anesthesia, such as tetracaine-adrenaline-cocaine or lidocaine-adrenaline-tetracaine gel or nerve block with 1% or 2% lidocaine. Very young or anxious patients may also require possible sedation with benzodiazepines, fentanyl, or ketamine.

While hemostasis is being achieved, a thorough history of the mechanism of injury should be assessed to determine wound contamination, presence of retained foreign bodies, and probable depth of injury. A tetanus immunization history should also be determined.

Closure of the wound may or may not be needed. Superficial scrapes and abrasions do not require closure. Deep lacerations that penetrate into or beyond the dermis may require direct closure with sutures or adhesives to provide an epithelial covering for healing by primary intention after thorough cleansing and debridement. Clean wounds can be closed within 8 hours of injury. Contaminated wounds typically should be left open if more than 6 - 8 hours old and allowed to heal spontaneously or by secondary intention, though late closure (tertiary intention) may be considered if no infection is evident. This usually includes puncture wounds or bite wounds.

You are evaluating a 32-year-old male who has possibly injured his Achilles tendon while planting his foot into the soft ground while playing touch football. When evaluating a patient with this suspected injury, you have the patient plantar flex his ankle as part of the exam. Based on your knowledge of anatomy, what two plantar flexing muscles attach into the Achilles tendon?

A Anterior tibialis and soleus
B Flexor hallucis longus and flexor digitorum
C Flexor hallucis longus and gastrocnemius
D Gastrocnemius and soleus
E Peroneus longus and peroneus brevis

Gastrocnemius and soleus
The gastrocnemius and soleus muscles form a common tendon at their distal insertions known as the Achilles tendon. This the strongest and thickest tendon in the body and it attaches at its distal end onto the calcaneus bone of the foot. Contraction of the gastrocnemius and soleus muscles are responsible for plantar flexion of the foot and ankle. The anterior tibialis muscle is one of the important dorsiflexors of the foot and ankle. Flexor hallucis longus and flexor digitorum are both involved in plantar flexion, but are not as strong as the gastrocnemius and soleus muscles and do not attach to the Achilles tendon. The flexor hallucis longus is primarily responsible for plantar flexion of the great toe and flexor digitorum produces plantar flexion of the remaining toes. The peroneus longus and peroneus brevis muscles do contribute to plantar flexion, but are not connected to the Achilles tendon. They are also the primary evertor muscles of the foot and ankle.

Which of the following joints has the lowest occurrence rate of osteoarthritis?

A Elbows
B Hands
C Hips
D Knees
E Spine

elbow
Because the elbow is not a weight bearing bone, the rate of osteoarthritis in the elbow is considerably less that what is found in locations like the hips, knees and spine. The hands have one of the highest rates of occurrence of osteoarthritis, likely due to their near constant use and propensity for minor (or major) injury. When elbow arthritis does develop it is often post-traumatic osteoarthritis related to a significant injury in the past that disrupted joint surface integrity or as a result of rheumatoid arthritis, a systemic illness. Osteoarthritis of the elbow will generally present with pain, stiffness, and decreased range of motion. Osteophytes that form on the medial elbow might be implicated should neurological symptoms develop that correlate with ulnar nerve distribution as this nerve does pass in close proximity to the elbow on the medial side.

A 70-year-old African American male was admitted to the hospital with complaints of a high grade fever, nausea, infection and severe back pain. Nearly two years prior while walking down his steps, he fell without incurring any apparent fractures. Since then he has been complaining of mild back pain, which worsened during cough. Lab reports reveal low red blood cell count but normal neutrophil and platelet count. The serum protein electrophoresis reveals the presence of paraprotein. Bone marrow biopsy reveals 50% infiltration by plasma cells. Based on this information, what would be your diagnosis?
Answer Choices
1 multiple myeloma
2 Waldenström's macroglobulinemia
3 monoclonal gammopathy of unknown significance (MGUS)
4 primary amyloidosis
5 polyclonal hypergammaglobulinemia

Multiple Myeloma
Explanation Multiple myeloma is a malignant disorder of the plasma cells. The disease occurs very frequently in people above 60 years of age. Males are more frequently affected than females. In the patients suffering from multiple myeloma, bone marrow biopsy shows heavy penetration with atypical plasma cells. Anemia is nearly universal in these patients. The hallmark of myeloma is the finding of a paraprotein on serum protein electrophoresis (SPEP). The majority of patients have a monoclonal spike in the beta or gamma globulin region. Overall, approximately 60% of myeloma patients have an IgG paraprotein, 25% have an IgA, and 15% have light chains only.
Monoclonal gammopathy of unknown significance (MGUS) closely matches multiple myeloma in the immunoelectrophoresis patterns. Thus, if one considers all patients with paraproteins, MGUS is far more common than myeloma. However, myeloma is distinguished from MGUS by findings of replacement of the bone marrow, bone destruction and progression over time. Although the height of paraprotein spike alone is not used to distinguish benign from malignant conditions. In practice all the patients with IgG spikes greater than 3.5g/dL prove to have myeloma. In polyclonal hypergammaglobulinemia there is a polyclonal instead of a monoclonal spike.

A 72-year-old woman presents with lower back pain and headaches that have progressively worsened over the last 6 months. She has recently noticed that she is more easily fatigued. Aside from tenderness in the lumbar spine, her physical exam is unremarkable. X-rays of the spine reveal scattered lytic lesions throughout the vertebrae, and a skull X-ray reveals a diffuse, "moth-eaten" appearance. Results from a blood count reveal a moderate, normocytic anemia; other values are within normal limits. The laboratory reports the presence of rouleaux in the peripheral blood smear. What test should be ordered next?

Answer Choices
1 A chest X-ray
2 A direct antiglobulin test
3 A serum protein electrophoresis
4 A bone scan
5 A test for rheumatoid factor

A serum protein electrophoresis

Explanation
Based on this woman's age, the lytic bone lesions, and the laboratory results, she is most likely suffering from multiple myeloma (MM), a malignancy of plasma cells.

Infiltration of the bone marrow by large numbers of these cells can lead to lytic bony lesions and pathologic fractures. Patients most commonly present with bone pain and anemia. Infections due to a dysfunctional immune system and evidence of some degree of kidney failure are also common.

The diagnosis of multiple myeloma is dependent on satisfying certain major and minor criteria. One of the major criteria can usually be satisfied by a serum protein electrophoresis, which demonstrates a monoclonal gammopathy in about 80% of patients with MM. The presence of more than 30% plasma cells on a bone marrow biopsy is another major criterion, although the test is obviously more invasive.

A chest X-ray would be unlikely to yield any useful additional information, and lytic lesions do not show up on bone scans, making that test unnecessary. It is unlikely that an autoimmune process is at work, so testing for rheumatoid factor or for antibodies attached to red blood cells (the direct antiglobulin test) would not prove useful.

A 9-month-old female from Southeast Asia infant presents to her pediatrician. She is suffering from failure to thrive, diarrhea, and recurrent fever. Upon examination, the physician notes that the infant is pale and has an enlarged abdomen due to hepatosplenomegaly. Blood analysis reveals a decreased mean corpuscular volume. Mean corpuscular hemoglobin and hemoglobin are also decreased at 15 pg and 6 g/dl, respectively. A peripheral smear shows microcytic, nucleated red blood cells; otherwise, the smear shows normal morphology and a few target cells. What is the most likely diagnosis?

Answer Choices
1 α-Thalassemia
2 β-Thalassemia
3 Hemophilia A
4 Sickle cell anemia
5 Von Willebrand disease

B thalassemia
Explanation
The correct answer is β-thalessemia. β-thalassemia is caused by mutations in the b-globin gene causing a reduced amount of b-globin to be produced. Individuals affected with b-thalassemia are normal at birth due to the presence of HbF. Anemia develops over the first few months, and symptoms become progressively more severe. It is characterized by failure to thrive, feeding difficulties, easy fatigue, bouts of fever and diarrhea, intestinal problems, and hepatosplenomegaly. Peripheral blood smear shows the presence of nucleated red blood cells. Mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH) are decreased; so is hemoglobin (<7 g/dl). Mutation analysis of the b-globin gene is available on a clinical basis.

α-thalassemia is caused by mutations in the alpha-globin genes, leading to decreased production of these polypeptide chains. 2 pairs of genes are involved, and inheritance is complicated. Symptoms range from no clinical abnormalities (a mutation in only 1 of the 4 genes) to lethal to the embryo (a mutation in each of the 4 genes).

Sickle cell anemia occurs almost exclusively in the African-American population; 1 in 400 African-Americans in the US are affected. It is characterized by anemia, growth retardation, developmental delay, and increased risk of infection. The altered red blood cells cause bloodflow to become sluggish and can cause occlusion of vessels. Jaundice may be present. Bone changes, which can be seen on X-ray, occur due to chronic marrow hyperactivity. Typical appearance includes a short trunk with long extremities and a tower-shaped skull. Aplastic crisis can occur when infection is present, and severe bone pain is common. Diagnosis is made by hemoglobin electrophoretic testing.

Hemophilia A and von Willebrand disease are bleeding disorders caused by deficiencies of different clotting factors: hemophilia A occurs when factor VIII is deficient; von Willebrand disease occurs when the von Willebrand factor is deficient or abnormal. They are clinically similar. They are characterized by prolonged or renewed bleeding after injuries, tooth extractions, or surgery. Coagulation factor assays are used for diagnosis. Hemophilia A is inherited in an X-linked recessive manner; only males are affected. Von Willebrand disease is inherited in an autosomal dominant manner.

A 58-year-old man presents with a 4-month history of worsening fatigue. The patient has noted "enlarged nodes" along the right side of his neck; he has never noticed these nodes before and states they are bothersome due to having so many; they also seem to be getting bigger. Physical examination reveals multiple cervical lymphadenopathies involving both the posterior cervical and the superficial cervical lymph nodes on the right side. Labs obtained the day of presentation include abnormalities, including a WBC count of 250,000/mm3 (normal: 5,000 - 10,000 mm3), lymphocytes being 77% of circulating WBCs (normal: 20 - 40%), and a smear revealing small but mature-appearing lymphocytes. A subsequent bone marrow biopsy reveals variably infiltrated small mature lymphocytes. Philadelphia chromosome is negative.

Question
Based on the history/physical examination and laboratory/diagnostic studies, what is the most likely diagnosis?

Answer Choices
1 Hairy cell leukemia (HCL)
2 Acute myeloid leukemia (AML)
3 Acute lymphoblastic leukemia (ALL)
4 Chronic lymphocytic leukemia (CLL)
5 Chronic myeloid leukemia (CML)

CLL
Explanation
The patient in the above scenario most likely has chronic lymphocytic leukemia (CLL). This is most likely due to the presenting symptoms, which include the patient's age (typically after 50 years of age, with a median age being 70), fatigue, and lymphadenopathy. Initial labs completed show an abnormally elevated white blood cell count with significant lymphocytosis; usually 75 - 98% of the cells are smaller (but mature-appearing) lymphocytes. Bone marrow biopsy is markedly infiltrated, with small (but mature) lymphocytes.

Hairy cell leukemia (HCL) is incorrect; lymphadenopathy is rarely found in this pathology with organomegaly found in almost all cases. Pancytopenia is present, and the evidence of 'hairy cells' are found on the peripheral blood smear, which are described as having cytoplasmic projections.

Acute myeloid leukemia (AML) is incorrect because symptoms of AML are usually only present for days or even weeks prior to the patient presenting for clinical evaluation. Symptoms include unusual bleeding from the skin and mucosal surfaces, bone/joint pain, evidence of petechiae and purpura. The hallmark AML laboratory finding is pancytopenia with circulating blasts; more than 20% marrow blasts are conclusive for the diagnosis of AML.

Acute lymphocytic leukemia (ALL) most often is characterized by a combination of pancytopenia with circulating blasts. The bone marrow biopsy will also be hyper-cellular and largely consisting of blasts. An amount of 20% or more blasts in the marrow is required to diagnose ALL; this is not consistent with the findings in the above scenario.

Chronic myeloid leukemia (CML) is the incorrect choice due to several factors: CML is typically a leukemia found in middle age patients with the median age at presentation being 55 years old. Patients will have fatigue, night sweats, a low-grade fever, and splenomegaly present. On very rare occasions, patients may also have blurred vision, respiratory distress, or even priapism. The hallmark finding of CML is the laboratory finding of the bcr/abl gene in the peripheral blood; this also known as the Philadelphia chromosome. The patient above is negative for this finding.

A 26-year-old woman presents with fatigue, malaise, headaches, blurred vision, and tinnitus. She denies any chest pain, abdominal pain, nausea, vomiting or diarrhea. She has not noticed any dark stools or abnormal urine. On exam she has a temperature of 100.3 F, pulse rate 102/min, BP 130/84, and SPO2 is 99%. Chest and abdominal exam are unremarkable, but the skin on her legs show ecchymoses.

Labs show Hb 8.8g/dl, WBC 11,000, and platelets 65,000. PT and PTT are normal. BUN is 16, creatinine is 1.1, and AST/AST are normal. UA shows 12 RBC with 5 WBC. Peripheral smear reveals fragmented red cells and LDH is 620 U/L.

What is the most likely diagnosis?

Answer Choices
1 Thrombotic thrombocytopenic purpura (TTP)
2 Hemolytic-uremic syndrome (HUS)
3 Idiopathic thrombocytopenic purpura (ITP)
4 Antiphospholipid antibody syndrome (APS)
5 Disseminated intravascular coagulation (DIC)

Thrombotic thrombocytopenic purpura
Explanation
TTP is a related condition that is characterized by microangiopathic hemolysis and platelet aggregationhyaline thrombi, whose formation is unrelated to coagulation system activity. It is also distinguished by the formation of hyaline thrombi in many organs, causing partial obstruction in the blood vessels. Von Willebrand factor is synthesized by the endothelial cells and built up into large multimers. The larger ones are called unusually large von Willebrand factor or ULWF. These are quickly broken down to the normal sized von Willebrand factor by a cleaving protease called ADAMTS-13, the deficiency of which, in TTP, leads to widespread accumulation of ULWF, platelet activation and aggregation, and formation of multiple thrombi.

TTP typically presents with the pentad of fever; microangiopathic hemolytic anemia; thrombocytopenia; neurological symptoms like tinnitus, blurred vision, headache, confusion, lethargy and seizures; and renal disease. Renal involvement is less severe than HUS and may be limited to hematuria or proteinuria, whereas neurological symptoms predominate unlike in HUS. The disease is fatal without treatment, which includes plasma exchange daily till neurological symptoms resolve and platelet count and LDH normalize. Other therapeutic measures include steroids, aspirin, immunoglobin, immunosuppressive agents, and splenectomy.

Hemolytic-uremic syndrome (HUS) is a primarily pediatric condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. In children it occurs after infection with toxin producing E. coli strain O157:H7 or shigella dysenteriae. In adults, sporadic cases occur after chemotherapy with mitomycin, cisplatin, bleomycin, or gemcitabine or with adenocarcinomas of breast GI tract, pancreas, or prostate. Renal function tests and peripheral smear (schistocytes are seen) are done. Therapeutic options include plasma exchange, dialysis, control of hypertension, low dose aspirin, and steroids. Prognosis remains poor, even with optimal treatment, since plasmapheresis appears to be ineffective in drug induced HUS. Discontinuing the offending drug is a must.

ITP, usually a diagnosis of exclusion, is an immune mediated thrombocytopenia caused by antibodies to platelets that presents with mucosal bleeding and petechiae. The acute form is seen in children after a viral illness, whereas the chronic form is commonest in young women, aged between 20-50 years. Adults present with purpura, menorrhagia, epistaxis, gingival bleeding, and bruising tendency. Low platelet count is found, sometimes severe, with increased megakaryocytes. All other lines of cells are usually normal. Therapy includes high dose steroids, immunosuppressive treatment with azathioprine, IVIG, or splenectomy. Platelet concentrate may be considered for transfusion.

APS is characterized by antibodies against phospholipids or plasma proteins bound to phospholipids and may be idiopathic or associated with SLE, rheumatoid diseases, infections, drugs, and neoplasms. It is associated with thrombotic events, fetal loss, thrombocytopenia, and neurological events. Renal disease of multiple etiology can occur. PT and PTT are prolonged. Treatment includes anticoagulation, antiplatelet therapy, and immunosuppressants.

DIC involves a complex series of events initiated by a systemic illness like sepsis, trauma, malignancy, abruptio placentae, etc, which manifests as a frequently catastrophic hematologic syndrome. Thrombosis and bleeding diathesis co-exist. Activation of the coagulation pathway causes thrombosis. Platelets and fibrinogen are consumed, causing bleeding. PT and PTT are prolonged. There is thrombocytopenia and increased fibrin degradation products. Therapy includes treating the underlying disease and administration of platelets and plasma to bleeding patients, whereas anticoagulation is reserved for thrombotic complications.

TTP is a related condition that is characterized by microangiopathic hemolysis and platelet aggregationhyaline thrombi, whose formation is unrelated to coagulation system activity. It is also distinguished by the formation of hyaline thrombi in many organs, causing partial obstruction in the blood vessels. Von Willebrand factor is synthesized by the endothelial cells and built up into large multimers. The larger ones are called unusually large von Willebrand factor or ULWF. These are quickly broken down to the normal sized von Willebrand factor by a cleaving protease called ADAMTS-13, the deficiency of which, in TTP, leads to widespread accumulation of ULWF, platelet activation and aggregation, and formation of multiple thrombi.

TTP typically presents with the pentad of fever; microangiopathic hemolytic anemia; thrombocytopenia; neurological symptoms like tinnitus, blurred vision, headache, confusion, lethargy and seizures; and renal disease. Renal involvement is less severe than HUS and may be limited to hematuria or proteinuria, whereas neurological symptoms predominate unlike in HUS. The disease is fatal without treatment, which includes plasma exchange daily till neurological symptoms resolve and platelet count and LDH normalize. Other therapeutic measures include steroids, aspirin, immunoglobin, immunosuppressive agents, and splenectomy.

A 29-year-old African-American man develops dysuria and increased frequency of micturition. In the emergency room, he is found to have a urinary tract infection and is treated with trimethoprim-sulfamethoxazole (Bactrim), as well as recommended to follow up with his primary care physician in the office. Over the next few days, the patient experiences fatigue, fever, jaundice, abdominal and back pain, and dark urine. Blood tests show Hb of 4g/dl, reticulocyte count of 6%, and MCV of 93. Peripheral smear reveals cell fragments, microspherocytes, and blister or bite cells. Heinz bodies are also present. Based on these findings, which clinical entity is most likely?

Answer Choices
1 Hereditary spherocytosis
2 Sickle cell anemia
3 Porphyria
4 G6PD deficiency
5 Autoimmune hemolytic anemia

G6PD deficiency
G6PD deficiency is a very common X-linked hemolytic disorder affecting millions across the world. The highest incidence is among people of African, Asian, or Mediterranean descent.

Glucose-6-phophate dehydrogenase is an enzyme in red cells that is vital to the integrity of the cell. Deficient individuals are susceptible to hemolysis under oxidant stress caused by drugs like sulfa and antimalarials, infections, or noxious agents like fava beans. Oxidant stress causes denatured hemoglobin, resulting in Heinz bodies. During an acute event, as in this patient, rapid hemolysis causes acute anemia, hemoglobinuria (thus the dark urine), abdominal and back pain, jaundice, and even renal failure. G6PD levels are normal to low during an acute attack, since the replacement reticulocytes are young and have adequate enzyme levels. G6PD level should be measured after an acute attack is over. Removing the precipitating agent and getting oxygen and rest is usually sufficient during an acute attack.

Hereditary spherocytosis is another type of hemolytic anemia due to congenital red cell membrane defect, causing red cell swelling and resulting in the typical small round spherocytes without any central pallor, as in a normal RBC. This results in increased osmotic fragility, and red cells are easily destroyed in the spleen. Symptoms vary from incidental diagnosis to severe anemia, to neonatal hyperbilirubinemia, to hydrops fetalis in utero. Patients may present with biliary symptoms in adulthood.

Sickle cell anemia is a hemoglobin disorder mostly occurring in African Americans in which there is a mutation in the amino acid sequence in the globin chains, resulting in severe structural and functional changes in the red cells. The hemoglobin molecule gets polymerized, resulting in rigidity of the molecule and causing red cells to have an altered shape resembling a sickle. This predisposes the red cells to undergo hemolysis with stressors like infections. Patient may have crises of different types, including aplastic, painful, hemolytic, etc. Gallstone disease may occur. Frequent visits to the ER are common with chest pain (acute chest syndrome), abdominal pain from splenic infarctions, and infections due to encapsulated organisms. Acute chest syndrome is defined as a new infiltrate on chest X-ray along with new symptoms such as cough, tachypnea, dyspnea, and fever. It is probably due to pulmonary infarction, due to sickle cells along with infection. Chest X-ray, sputum cultures, and blood cultures are requested. Treatment is mainly supportive with oxygen, appropriate antibiotics, fluid management, chest physiotherapy, and incentive spirometry.

Labs show chronic anemia with Hb of 5-10g/d. WBC count, arterial blood gases, and liver function tests are done. Electrophoresis can confirm diagnosis. Parvovirus infection induces aplastic crisis. Oxygen, analgesia, and blood transfusions are necessary to treat the patient.

Porphyria is an autosomal dominant disease due to genetic or acquired deficiencies in the activity of enzymes in the heme biosynthetic pathway. Symptoms include abdominal pain, dark urine, and neurological and psychiatric manifestations. UA shows high porphyrin content.

Autoimmune hemolytic anemia occurs due to development of antibodies directed at red cells, resulting in their destruction. It occurs in patients with systemic autoimmune disorders like SLE and ulcerative colitis, lymphoproliferative disorders like lymphomas, and in AIDS. Steroids and splenectomy may be needed to treat this condition. Patients present with anemia, jaundice fever, abdominal pain, and dark urine. Labs show anemia with normal RBC indices, increased reticulocyte count, nucleated red cells and spherocytes in the peripheral smear, and erythroid hyperplasia in the bone marrow. Coomb's test may be positive. Transfusion may be life saving.

A 5-year-old African-American girl with sickle cell disease presents due to right leg pain. 2 weeks ago, she began to experience right thigh pain and a slight limp following a playground injury. Upon sustaining the injury, she was taken to a local ER; X-rays were negative for evidence fracture. She was diagnosed with and treated for a leg contusion. Over the past 2 weeks, the pain has become more severe, and she has experienced fever; temperatures have been as high as 102°F, and she has experienced episodic chills.
On physical examination, her temperature is 101°F; her right leg is swollen, tender, and erythematous over the anterior aspect of the thigh. The remainder of the PE is unremarkable. CBC with differential reveals a leukocytosis; there is a predominance of neutrophils and a bandemia.
Question
What is the most appropriate empiric treatment?
Answer Choices
1 Ceftriaxone and Vancomycin
2 Gentamicin and Vancomycin
3 Nafcillin and a Fluoroquinolone
4 Nafcillin and Vancomycin
5 Vancomycin and a Fluoroquinolone

Ceftriaxone and Vancomycin
Explanation
The correct response is ceftriaxone and vancomycin. This patient most likely has hematogenous osteomyelitis. Osteomyelitis refers to inflammatory destruction of bone due to infection. There are 2 main categories of osteomyelitis:
Hematogenous osteomyelitis, which is most commonly found in children and occurs secondary to sepsis
Osteomyelitis directly resulting from the spread of bacteria from an adjacent infection (infected diabetic foot ulcer), trauma (open fractures), or vascular insufficiency (peripheral vascular disease). Osteomyelitis resulting from bacteremia is a disease most frequently associated with sickle cell disease, injection drug users, diabetes mellitus, and the elderly. Patients with this form of osteomyelitis often present with sudden onset of high fever, chills, pain, and tenderness of the involved bone.
Among patients with hemoglobinopathies such as sickle cell disease, osteomyelitis is caused most often by Salmonella paratyphi. In sickle cell disease, as well as with the other hemoglobinopathies, Salmonella osteomyelitis is 10 times more common than osteomyelitis caused by other organisms. Staphylococcus aureus is the 2nd most common cause of osteomyelitis in SCD patients. Any empiric antibiotic regimen will need to provide adequate cover for both of these organisms.
A 3rd-generation cephalosporin, such as ceftriaxone or cefotaxime, plus an antistaphylococcal agent (vancomycin or clindamycin and nafcillin) is a reasonable choice; the treatment will provide coverage for both Salmonella and Staphylococcus. If this child had acquired the infection while in the hospital, vancomycin or clindamycin would be the most appropriate empiric antistaphylococcal coverage, not nafcillin, because of the increased prevalence of hospital-acquired infection with methicillin-resistant S. aureus (MRSA).
Nafcillin and Vancomycin is incorrect. This combination covers for MRSA, but not Salmonella.
Fluoroquinolone should not be used in this case. Current evidence in the medical literature has demonstrated an increase in the emergence of fluoroquinolone-resistant Salmonella paratyphi species.
Gentamicin and Vancomycin is incorrect. Gentamicin is an aminoglycoside most effective against Gram-negative bacteria.
Chloramphenicol has historically been most effectively against Salmonella osteomyelitis in SCD. It is not widely used systemically today due to toxicity; however, it does still have a place in severe salmonella infections, and it could be a viable empiric choice along with vancomycin in this case.

A 48-year-old previously healthy African American woman was involved in a severe motor vehicle accident, sustaining multiple injuries. She was stabilized in the Emergency Department, but is now bleeding extensively from her laceration sites, her IV catheter site, and from mucous membranes. Laboratory results show thrombocytopenia, fragmented red blood cells, and low fibrinogen levels. What is this patient's most likely diagnosis?
Answer Choices
1 Factor V deficiency
2 Disseminated intravascular coagulation
3 Factor XI deficiency
4 Protein C and S deficiency
5 Acute idiopathic thrombocytopenia purpura

Disseminated intravascular coagulation
Explanation This patient's severe injuries triggered the mechanisms of disseminated intravascular coagulation (DIC). In DIC, traumatized or necrotic tissue releases tissue factor into the circulation, which triggers coagulation reactions. Thrombi and emboli develop. The thrombotic phase is followed by the fibrinolysis. Hemorrhage results from depletion of coagulation factor and platelets. Diagnosis of DIC is made by thrombocytopenia, fragmented red blood cells, prolonged PT and PTT, decreased fibrinogen, and increased fibrin degradation products.
Though Factor V deficiency can manifest after trauma, this condition is very rare and usually not this severe.

Factor XI deficiency is more common in Ashkenazi Jews. Patients with this disease may have post-traumatic bleeding, but spontaneous bleeding is less common.

With protein C and S deficiency, patients experience recurrent venous thrombi and pulmonary emboli, not bleeding.

Acute idiopathic thrombocytopenia purpura (ITP) is much more common in children, especially following a viral exanthem. Acute ITP is rare in adults.

A 55-year-old man presents with fatigue, night sweats, and low-grade fever of 1 week's duration. On physical examination, you note a palpable spleen. You order a CBC, and the results indicate a white blood count of 105,000 cells/mcL with a left shift of the myeloid series. The red blood cell count and morphology are unremarkable, and he has a slightly elevated platelet count. To help confirm your suspicions, you order genetic studies, and the results come back with the BCR/ABL gene detected.
Question
What is the most likely diagnosis?

Answer Choices
1 Chronic lymphocytic leukemia
2 Acute lymphocytic leukemia
3 Chronic myelogenous leukemia
4 Acute myelogenous leukemia
5 Polycythemia vera

Chronic myelogenous leukemia
Explanation
The clinical picture is suggestive of chronic myelogenous leukemia (CML). CML is a myeloproliferative disorder characterized by an overproduction of myeloid cells. Depending on disease progression, common findings include a strikingly high WBC (usually >100,000 cells/cmm), normal RBC count, and RBC morphology is usually normal, although nucleated RBCs may sometimes be present. The platelet count may be normal or elevated. A left shift of myeloid cells is noted. Definitive diagnosis is made by finding the BCR/ABL gene.

CML has 3 stages: chronic, accelerated, and blastic. Symptoms, such as fever, night sweats, and tiredness, can appear at any point in the chronic phase.

Chronic lymphocytic leukemia will have predominantly mature lymphocytes noted on the CBC, which is not seen in this patient.

The acute leukemias will have predominantly blasts noted on the morphology review.

Polycythemia vera will have elevated WBC, RBC, and platelet counts. Key finding is a hematocrit > 60%.

A 63-year-old man presents with bleeding gums and a general feeling of illness. He indicates that he has been running a low-grade fever for the past few days; he feels fatigued, and he has bone and joint pain. He thought that he had the flu, but he became concerned when his gums began to bleed. Physical examination reveals a pale individual, mild petechia on the extremities, gingival hyperplasia, and a palpable spleen. A CBC reveals pancytopenia and blasts with Auer rods on the differential.

Question
What is the most likely diagnosis?

Answer Choices
1 Chronic lymphocytic leukemia (CLL)
2 Acute myelogenous leukemia (AML)
3 Multiple myeloma
4 Chronic myelogenous leukemia (CML)
5 Polycythemia vera

Acute myelogenous leukemia (AML)
Explanation
The clinical picture is suggestive of acute myelogenous leukemia (AML). AML is a myeloproliferative disorder affecting older individuals; the median age is 60 years old. Most arise without any clear cause, but many chemical agents and radiation are linked to causing AML. Features of this disorder include short duration of fever, fatigue, and bleeding. The CBC will reveal pancytopenia or cytopenia. Blasts can be seen in the peripheral smear in 90% of patients. Cytoplasmic inclusion bodies called Auer rods are pathognomonic of this disorder.

CLL is characterized by isolated lymphocytosis, which is not seen in this patient.

Multiple myeloma is a malignancy of plasma cells, which is not described in this patient.

CML is characterized by a very high WBC count (>100,000/mcL), which is not seen in this patient.

Polycythemia vera is characterized by overproduction of all 3 hematopoietic cell lines, primarily the RBC cell line.

Defective red cell membrane protein (spectrin) deficiency is seen in which of the following hemolytic anemias?
Answer Choices
1 Congenital spherocytosis
2 Thalassemia
3 Sickle cell anemia
4 Paroxysmal nocturnal hemoglobinuria
5 Hemoglobin C disease

Explanation Hereditary spherocytosis is an autosomal dominant disorder characterized by deficiency of a red cell membrane protein known as spectrin. Patients with hereditary spherocytosis suffer from anemia and recurrent hemolysis. Bone marrow shows compensatory hyperplasia. Sequestration of spherocyte by the spleen results in splenomegaly.

A 19-year-old woman presents with weakness and increased fatigue over the past several months. Laboratory studies reveal haemoglobin of 9.7g/dL with a haematocrit of 32%.

Question
Which of the following results would indicate an iron deficiency anemia?
Answer Choices
1 Increased TIBC, decreased serum ferritin, decreased serum iron
2 Decreased TIBC, decreased serum ferritin, decreased serum iron
3 Increased TIBC, increased serum ferritin, increased serum iron
4 Increased TIBC, increased serum ferritin, decreased serum iron
5 Decreased TIBC, increased serum ferritin, decreased serum iron

Increased TIBC, decreased serum ferritin, decreased serum iron
Explanation The correct answer is increased Total Iron Binding Capacity (TIBC), decreased serum ferritin, and decreased serum iron. In patients with iron deficiency anemia, the first iron stores that are depleted can be indicated by a decreased ferritin, causing the TIBC to rise. This type of anemia is described as hypochromic and microcytic due to the decreased iron stores present. Red blood cell formation continues but in the presence of decreased iron supply. The mean corpuscular volume (MCV) will stay normal early in the disease process but will fall over time.

The other answers have results that do not represent iron deficiency anemia.

A 28-year-old female, with no particular past medical history, notices a rash on both her thighs as she is changing clothes one morning. She delivered a baby by C-section approximately 3 months ago. Since then she has had a fairly unremarkable post partum course, except for a respiratory infection 3-4 weeks ago, for which she received sulfamethoxazole/trimethoprim (Bactrim DS). She is no longer nursing. On exam her temperature in 98.4, pulse rate 80/min, BP 110/70, and SPO2 92%. Oral exam reveals two small hematomas on her tongue and one on the inside of her lower lip. Her thighs show diffuse petechiae bilaterally. Chest and abdominal, as well as neurological, exam is normal.
Labs show Hb 12g/dl, WBC 7400, and platelets 6000. MCV is 92, PT/PTT are normal, BUN 14, creatinine 0.9, AST/ALT normal, and UA shows 2 WBC and 0 RBC. Peripheral smear was non contributory. Patient is empirically started on steroids at 80mg daily, and platelets are checked every third day. The number goes up to 96,000 over the next few weeks. In all probability, the patient has:

Answer Choices
1 Thrombotic thrombocytopenic purpura
2 Hemolytic-uremic syndrome
3 Idiopathic thrombocytopenic purpura
4 Antiphospholipid antibody syndrome
5 Disseminated intravascular coagulation (DIC)

Idiopathic thrombocytopenic purpura
Explanation Hemolytic-uremic syndrome (HUS) is a primarily pediatric condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. In children it occurs after infection with toxin producing E. coli strain O157:H7 or shigella dysenteriae. In adults, sporadic cases occur after chemotherapy with mitomycin, cisplatin, bleomycin, or gemcitabine, or with adenocarcinomas of breast GI tract, pancreas, or prostate. Renal function tests and peripheral smear (schistocytes are seen) are done. Therapeutic options include plasma exchange, dialysis, control of hypertension, low dose aspirin, and steroids. Prognosis remains poor even with optimal treatment, since plasmapheresis appears to be ineffective in drug induced HUS. Discontinuing the offending drug is a must.
TTP is a related condition that is characterized by microangiopathic hemolysis and platelet aggregationhyaline thrombi, whose formation is unrelated to coagulation system activity. It is also distinguished by the formation of hyaline thrombi in many organs, causing partial obstruction in the blood vessels. Von Willebrand factor is synthesized by the endothelial cells and built up into large multimers. The larger ones are called unusually large von Willebrand factor or ULWF. These are quickly broken down to the normal sized von Willebrand factor by a cleaving protease called ADAMTS-13, the deficiency of which, in TTP, leads to widespread accumulation of ULWF, platelet activation and aggregation, and formation of multiple thrombi.

TTP typically presents with the pentad of fever, microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms like tinnitus, blurred vision, headache, confusion, lethargy and seizures, and renal disease. Renal involvement is less severe than HUS and may be limited to hematuria or proteinuria, whereas neurological symptoms predominate unlike in HUS. The disease is fatal without treatment, which includes plasma exchange daily till neurological symptoms resolve and platelet count and LDH normalize. Other therapeutic measures include steroids, aspirin, immunoglobin, immunosuppressive agents, and splenectomy.

ITP, usually a diagnosis of exclusion, is an immune mediated thrombocytopenia caused by antibodies to platelets that presents with mucosal bleeding and petechiae. The acute form is seen in children after a viral illness, whereas the chronic form is commonest in young women, aged between 20-50 years. Adults present with purpura, menorrhagia, epistaxis, gingival bleeding, and bruising tendency. Low platelet count, sometimes severe, is found with increased megakaryocytes. All other lines of cells are usually normal. Therapy includes high dose steroids, immunosuppressive treatment with azathioprine, IVIG, or splenectomy. Platelet concentrate may be considered for transfusion.

APS is characterized by antibodies against phospholipids or plasma proteins bound to phospholipids and may be idiopathic or associated with SLE, rheumatoid diseases, infections, drugs, and neoplasms. It is associated with thrombotic events, fetal loss, thrombocytopenia, and neurological events. Renal disease of multiple etiology can occur. PT and PTT are prolonged. Treatment includes anticoagulation, antiplatelet therapy, and immunosuppressants.

DIC involves a complex series of events initiated by a systemic illness like sepsis, trauma, malignancy, abruptio placentae, etc., which manifests as a frequently catastrophic hematologic syndrome. Thrombosis and bleeding diathesis co-exist. Activation of the coagulation pathway causes thrombosis. Platelets and fibrinogen are consumed, causing bleeding. PT and PTT are prolonged. There is thrombocytopenia and increased fibrin degradation products. Therapy includes treating the underlying disease and administration of platelets and plasma to bleeding patients, whereas anticoagulation is reserved for thrombotic complications.

A 56-year-old right hand dominant male presents with swelling to the right elbow. He explains to you that he has had this type of swelling before and was diagnosed with bursitis. Based on this description, where would the most likely site for the elbow bursitis be located?

A Capitellum
B Lateral epicondyle
C Medial epicondyle
D Olecranon
E Radial head

Olecranon
The olecranon bursa is very vulnerable to injury and chronic inflammation due to its superficial location on the extensor side of the elbow. Acute falls, chronic pressure, and other inflammatory processes such as rheumatoid arthritis or gout may contribute to olecranon bursitis. The medial and lateral epicondyles are known to be associated with chronic tendonopathies. The radial head is a common location for subluxation or fracture, while the capitellum at the distal end of the humerus is sometimes associated with fractures and articular cartilage damage.

The term "nursemaid's elbow" refers to which of the following physical conditions?

A A fracture of the humerus
B A subluxation of the radial head
C Inflammation at the lateral epicondyle
D Inflammation of the medial epicondyle
E Olecranon bursitis

a subluxation of the radial head
This is the most common elbow injury in children under the age of 5. The injury generally occurs when the child's arm is forcefully pulled when the elbow is in an extended position and the forearm is pronated. Fibers of the annular ligament that encircle the radial neck become trapped between the radius and ulna. On presentation, children hold their arm in slight flexion and pronation. There is no fracture associated with nursemaid's elbow and the olecranon bursa is unaffected. Nursemaid's elbow is an acute injury, while medial and lateral epicondylitis (also known as golfer's elbow and tennis elbow respectively) are generally chronic conditions.

Which of the following clinical manifestations is most characteristic of polymyalgia rheumatica (PMR)?

A subcutaneous inflammatory lesions
B pain and stiffness of proximal muscle groups
C insidious onset of symmetrical joint involvement
D widespread musculoskeletal pain and tender points
E symmetrical weakness initially in the legs that progresses caudally

pain and stiffness of proximal muscle groups
An abrupt onset of proximal muscle pain and stiffness in the shoulder and pelvic girdle areas, usually associated with fever, malaise, and weight loss, is characteristic of polymyalgia rheumatica. Subcutaneous inflammatory lesions denote erythema nodosum. These lesions are associated with pregnancy and several systemic disorders, such as sarcoidosis, tuberculosis (TB), and streptococcal infections. Insidious onset of symmetrical joint involvement is most commonly associated with rheumatoid arthritis. Widespread musculoskeletal pain and tender points, referred to as "trigger points," are seen with fibromyalgia syndrome. Trigger points may be found anywhere on the body but are most common in the neck, shoulders, hands, low back, and knees. Symmetrical weakness initially in the legs that progresses caudally is characteristic of Guillain-Barré syndrome

An avulsion fracture at the base of the fifth metatarsal is commonly called which of the following?

A Bennett fracture
B Boxer's fracture
C Chauffer's fracture
D Jones fracture
E Lisfranc fracture

Jones fracture
An avulsion fracture at the base of the fifth metatarsal, usually secondary to plantar flexion and inversion is called a Jones fracture. Also called a ballet or dancer's fracture, it is the most common metatarsal fracture. The fracture occurs at the proximal diaphysis. A Bennett fracture is an oblique fracture of the first metacarpal near the carpometacarpal joint. A boxer's fracture is a fracture of the fifth metacarpal. This is the most common fracture of the hand. A chauffer's fracture is an oblique fracture through the base of the radial styloid in the forearm. A Lisfranc fracture is actually a fracture and dislocation involving the tarsometatarsal joints.

A 21 year-old female presents to the emergency department after having a cast applied to her right arm earlier that day. Approximately one hour ago she began having extreme 10/10 pain in her right arm and is in visible distress. When considering the diagnosis of compartment syndrome, permanent damage to the muscle begins after how many hours of ischemia?

A >2 hours
B >6 hours
C >8 hours
D >12 hours
E >24 hours

>8 hrs
Permanent damage results after >8 hours (C) of ischemia. Nerves begin to lose conduction within 2 hours of onset of elevated pressures. Neurapraxia can occur within 4 hours, and irreversible damage occurs 8 hours after elevated pressures. Functional impairment is unlikely when compartment syndrome is diagnosed and treated within 6 hours of its onset. While deficits can occur prior to 8 hours, permanent damage is usually not seen before 8 hours (A and B). After 12 or 24 hours, permanent damage has already resulted in most cases (D and E). The key to early detection of compartment syndrome is a high index of clinical suspicion.

Which of the following is one standard of care to treat a first-time deep vein thrombosis (DVT) without pulmonary embolism (PE)?

A Begin the patient on warfarin 5 mg PO once daily and check an INR in five days and adjust to therapeutic levels continuing warfarin for six months.
B Begin the patient on enoxaparin 1 mg/kg SQ QD while also starting warfarin 5 mg PO once daily and check INR in five days and adjust to therapeutic levels continuing warfarin for six months and enoxaparin until therapeutic on the warfarin.
C Begin the patient on enoxaparin 2 mg/kg SQ QD while also starting warfarin 5 mg PO once daily and check INR in five days and adjust to therapeutic levels continuing warfarin for six months and enoxaparin until therapeutic on the warfarin.
D Begin the patient on heparin sodium IV and monitor and adjust levels to achieve therapeutic levels Q six hours and transition to warfarin as soon as possible.
E Initiate a hypercoaguability work up and treat accordingly.

Begin the patient on enoxaparin 2 mg/kg SQ QD while also starting warfarin 5 mg PO once daily and check INR in five days and adjust to therapeutic levels continuing warfarin for six months and enoxaparin until therapeutic on the warfarin.
Patients with deep vein thrombosis (DVT) without pulmonary embolism (PE) do not necessarily require hospitalization. Outpatient therapy would include treatment with a low molecular weight heparinoid (LMWH), such as enoxaparin, subcutaneously while the patient becomes therapeutic on oral warfarin. The LMWH can be discontinued once therapeutic on warfarin, with continued monitoring of INRs for six months before considering discontinuation.

LMWH= Enoxaparin (lovenox), Dalteparin sodium (fragmin), Tinzaparin (Innhep)

A 72-year-old male presents with bony pain, Bence Jones protein in his urine, an elevated creatinine level of 2.0, hypercalcemia, and lytic lesions to the long bones of his legs. The best initial treatment to correct this patient's renal insufficiency is which of the following?

A Bisphosphonate and plasma phoresis
B Dialysis and calcitonin
C Hydration and calcitonin
D Hydration and rapid single infusion of bisphosphonate
E Plasmaphoresis and hydration

Hydration and calcitonin
Management of renal impairment in patients with multiple myeloma is primarily supportive care. Hydration and use of calcitonin is the mainstay. If the hypercalcemia needs rapid correction, a slow infusion of bisphosphonate may be used. Dialysis would be useful if the patient has renal failure but has difficulty removing light chains from the blood, which are causing the problem. Plasmaphoresis doesn't play a role in this setting.

A patient that has exhibited excessive bleeding tendencies is scheduled for a cholecystectomy. By history, he is noted to be of Ashkenazi Jewish decent. Which coagulation disorder is most likely the cause of his bleeding tendency?

A Factor V
B Factor VII
C Factor VIII
D Factor IX
E Factor XI

Factor XI
Most patients with factor XI deficiency are Jewish, with most in the Ashkenazi population. While other factors may affect individual patients, the most common of these coagulation disorders within this population base is factor XI.

In a person with normal marrow function what is the mean life span of platelets?

A 3 to 6 days
B 7 to 10 days
C 11 to 14 days
D 15 to 18 days
E 19 to 22 days

7 to 10 days
Under normal conditions, human platelets have a mean life span in circulation of 7 to 10 days. In order to confirm that the thrombocytopenia is only related to the surgery, waiting at least a week for full platelet conversion is needed.

A 24-year-old male with thalassemia major who has received adequate transfusions, chelation therapy, and regular health checkups is in to establish care. He is 5'4" with a BMI of 17. He eats a balanced healthy diet and gets regular exercise. You know that there are complications of this disease. What is the best next step for this patient?

A Increase threshold for blood transfusions
B Maintain sustained reduction of body iron
C Obtain a Dexa scan for osteoporosis
D Obtain an ECHO to evaluate for cardiac siderosis
E Obtain regular testosterone levels and treat

Maintain sustained reduction of body iron
Maintaining sustained reductions in body iron has demonstrated increased overall survival rates through reductions in cardiac disease specifically due to siderosis. While these patients are at increased risk for osteoporosis and cardiac siderosis, the next best step in this patient is to maintain reduced iron levels. There is no place for increased blood transfusion or obtaining regular testosterone levels.

A 36-year-old male with a history of acute lymphoblastic leukemia (ALL) is treated with high dose methotrexate and glucocorticoids, vincristine, and L-asparaginase at age 10. He has been in remission since induction therapy was completed. He presents with pain to his right hip/back area, which radiates down his leg. He is also having some back pain that keeps him up at night. What is the most likely diagnosis?

A Bladder cancer with referred pain
B Lymphoma with metastasis to the bone
C Osteonecrosis of the right hip
D Osteoporosis
E Relapsed ALL

Osteonecrosis of the right hip
Common treatment sequelae that can present years out from therapy include osteonecrosis, especially following the use of high dose methotrexate and glucocorticoids. Lymphoma can arise as a post transplant complication, but is not typically seen following anti-leukemic therapy. Osteoporosis is also a complication of anti-leukemic therapy, but is more associated with the use of mercaptopurine. Relapsed ALL is typically seen in the first two years following therapy, but can arise more than 10 years out. Typically, this is seen with fever, pancytopenia, and bony pain. The pain is not typically isolated to one side of the body.

A 64-year-old male presents with fatigue, pallor, and dyspnea on exertion. He has noted this gradual onset over the last several months. His initial reason for coming in was due to new papules and nodules that he noted on his skin, with some violaceous in color. On exam you also note petechiae to the lower extremities and splenomegaly. On questioning him further, he notes feeling full sooner than he had in the past. On laboratory you note a pancytopenia. What is the most likely diagnosis?

A Acute myelogenous leukemia with leukemia cutis
B Hereditary Spherocytosis with drug eruption
C Lymphoma with erythema nodosum
D Pyoderma Gangrenosum
E Sarcoidosis

Acute myelogenous leukemia with leukemia cutis
The most likely diagnosis is AML with leukemia cutis. Leukemia cutis is seen in a subset of AML patients that are nontender, and are infiltrates of leukemic cells into the dermis. Hereditary spherocytosis is a hemolytic anemia, and does not present with papules and nodules or pancytopenia. Lymphoma does not typically present with a pancytopenia or skin rash. Pyoderma gangrenosum is an uncommon ulcerative cutaneous condition of uncertain etiology. Sarcoidosis is a chronic noncaseating granulomatous disease of unknown etiology, which affects many organs and tissues, most commonly the lungs.

A 23-year-old male presents with uncontrolled mucocutaneous bleeding following a wisdom tooth extraction. Traditional methods of controlling the bleeding have not significantly reduced the bleeding. On history, he reports epistaxis that takes longer than usual to stop bleeding. He also states that he seems to bruise more frequently than his friends. What laboratory test would be the most beneficial given his history?

A Bleeding time and platelet count
B Bleeding time and VWF antigen
C Factor VII assay and factor VIII assay
D Factor VII assay and platelet count
E Factor VIII assay and VWF antigen

Factor VIII assay and VWF antigen
In a patient suspected of having von Willebrand's disease, routinely performed tests include the assay of factor VIII activity, VWF antigen, and VEW Ristocetin cofactor activity. Bleeding time was previously used as a screening test, but is inconsistent. Factor VII assay would be used for someone suspected of having VII deficiency. Platelet counts are usually normal in a patient with von Willebrand's disease.

A 13-year-old female is seen for the first time to establish care. She is known to carry the diagnosis of beta-thalassemia major and has been maintained on regular transfusions for her anemia. She is short for her age and has not achieved menarche. Her glucose level is elevated and she has developed signs of diabetes mellitus. These findings are consistent with which of the following?

A End stage thalassemia
B Inadequate iron chelation
C Inadequately transfused anemia
D Stage 3 thalassemia
E Untreated thalassemia

Inadequate iron chelation
Inadequate iron chelation in patients with thalassemia major results in the absence of a pubertal growth spurt and failure of menarche. These patients may also develop diabetes mellitus, as well as other endocrine disturbances. There is no staging to thalassemia, and untreated or inadequately transfused anemia results in increased infections early in life, usually causing death, spontaneous fractures, and other deformities. Additionally, thrombocytopenia and leucopenia may develop.

A 38-year-old thin, HIV+ male is found to have a distended abdomen and is complaining of a feeling of fullness. A CT scan of the abdomen revealed a large abdominal mass with ascites in the retroperitoneal region. Laboratories found an elevated EBV of 5400 copies (normal=0), LDH 554 (100 to 250), and Ig-positive MYC translocation. What is the most likely diagnosis?

A Burkitt lymphoma
B Cat scratch disease
C Hodgkin's lymphoma
D Infectious mononucleosis
E Sarcoidosis

Burkitt's Lymphoma
In immunosuppressed individuals, including those who are HIV+ and in the presence of highly reactive EBV, the development of an abdominal mass with elevated LDH is most likely Burkitt's lymphoma. Of the masses that are found, 30 to 40% will be positive for EBV, and it appears that this virus drives the conversion of cells to a cancerous state. Infectious mononucleosis would be positive for EBV, but does not develop large abdominal masses.

A 57-year-old male is being monitored for Binet Stage A CLL. He is emergently seen in the clinic with rapid lymph node enlargement, fever, weight loss, and hepatosplenomegaly. On laboratory examination, he is found to have an elevated serum lactate dehydrogenase and a monoclonal gammopathy on serum protein electrophoresis. A retroperitoneal ultrasound reveals bulky adenopathy. What is the most likely diagnosis?

A Acute lymphadenitis secondary to HSV
B Epstein-Barr viral infection
C Hodgkin lymphoma
D Rapidly advancing CLL
E Richter transformation

Richter transformation
All findings are classic for Richter transformation of CLL to an aggressive large B-cell, high-grade lymphoma. While HSV cannot be totally ruled out without a node biopsy, given the patient history and findings the most likely diagnosis is Richter transformation. Rapidly advancing CLL does not usually develop retroperitoneal adenopathy. Occasionally, Richter syndrome with Hodgkin lymphoma features is seen, but accounts for less than one-fifth of all cases of Richter transformation. EBV may play a role in this later syndrome.

A 42-year-old African American male is admitted to the hospital with heme positive urine and anemia. He recently completed a course of trimethoprim/sulfamethoxazole for a urinary tract infection. Treatment for his condition should include which of the following?

A Begin vancomycin 1 GM IV q 12 for untreated infection
B No treatment
C Restart bactrim IV for an undertreated UTI
D Transfuse packed red cells
E Transfuse platelets

No treatment
No treatment is necessary, except to avoid known oxidant drugs. The patient is not exhibiting signs of an untreated infection, so A is wrong. The anemia is transient so no transfusions are needed.

An Rh-negative, 5-year-old male child presents with acute onset of petechiae and purpura after an acute viral illness. In addition, he has episodes of epistaxis. Which of the following is a treatment option if his platelet count falls below 20,000/mm 3 , but he is not actively bleeding?

A platelet transfusions
B IV anti-D (WinRho SD) 50-70 mg/kg/dose
C prednisone 2.4 mg/kg/24 hours × 2 weeks
D splenectomy

Prednisone 2.4 mg/kg/24 hours × 2 weeks
In patients with idiopathic thrombocytopenic purpura, treatment options should be initiated when platelet counts fall below 20,000, regardless of whether there is active bleeding or not. Without active bleeding the treatment options include prednisone 2-4 mg/kg/24 hours for 2 weeks; IV immunoglobulin 1 g/kg/24 hours for 1 to 2 days, or IV anti-D 50-75 μg/kg/dose for Rh-positive patients. Splenectomy is indicated for life-threatening bleeding. There is currently no indication for platelet transfusion and none of the above treatments are considered optimal, because in the majority of children, it will resolve on its own within 6 months.

A 50-year-old male with an elevated WBC of 143,000, hematocrit 47% (45 to 52%), platelet count of 247,000 (150,000 to 4000,000), and BCR/ABL found in peripheral blood. He complains of fatigue, night sweats, and abdominal fullness. The best initial course of therapy would include which of the following?

A Bone marrow transplant
B Cytarabine
C Dasatinib
D Hydroxyurea
E Imatinib mesylate

Imatinib mesylate
This patient has classic signs of chronic myelogenous leukemia. The first line of therapy for patients in the chronic phase of this disease is Imatinib mesylate, starting at 400 mg/day. For patients that demonstrate to Imatinib alone, Dasatinib may be an adjunct therapy. Hydroxyurea and Cytarabine were used prior to formulation of Imatinib, and may play a role for resistant CML. Bone marrow transplant would be reserved for patients that are resistant to current therapy or are in relapse.

A patient that was diagnosed with severe hemophilia B in 1974 and requiring regular factor IX replacement therapy will have a 1:2 chance of also having which of the following?

A Hemarthroses
B Hemophilia A
C Hepatitis B
D Hepatitis C
E HIV

HIV
Approximately 50% of older and severely affected patients with either hemophilia A or hemophilia B are now HIV positive, due to the pooled nature of therapeutic products prior to 1985. These patients are also at increased risk for Hepatitis B and C, but not at the same ratio. Hemarthroses is a complication of not treating factor IX bleeding.

A 27-year-old African American with sickle cell anemia presents to the emergency department with acute onset intractable pain. She is taking quick, shallow breaths and her oxygen saturation is 84% on room air. She appears desiccated, states she hasn't eaten in the last 24 hours, and says that she "just doesn't feel well." She is also afebrile. What should your next course of action be?

A Start morphine, hydrate, and start antibiotics
B Start morphine, oxygen, and start antibiotics
C Start oxygen, hydrate, and exchange transfusion
D Start oxygen, hydrate, and give pneumococcal vaccination
E Start oxygen, hydrate, and start antibiotics

Start oxygen, hydrate, and exchange transfusion
Exchange transfusions are primarily indicated for the treatment of intractable pain crises, priapism, and stroke. Patients should be kept well hydrated, and oxygen should be given if the patient is hypoxic. Antibiotics would be used if there was an infection identified, but are not part of initial treatment in a sickle crises.

A patient being treated for Burkitt's lymphoma is found to have hyperkalemia, hyperphosphatemia, hypocalcemia, a metabolic acidosis, hyperuricemia, and appears to be going into renal failure after the first dose of chemotherapy. These are all classic signs of which of the following?

A Chemo-reactive disease
B Chemotherapeutic anaphylaxis
C Pseudohyperkalemia syndrome
D Refeeding syndrome
E Tumor lysis syndrome

tumor lysis syndrome
Tumor lysis syndrome is a serious metabolic complication of rapidly growing tumors. Burkitt's is a classic example of a rapidly growing tumor that can cause this syndrome, as the tumor is broken down by the chemotherapeutic treatment. Chemo-reactive disease means that the tumor responds to chemotherapy. Anaphylaxis is an immune mediated response to any allergen producing substance. There is no diagnosis called Pseudohyperkalemia syndrome. Refeeding syndrome is caused by patients that haven't eaten for an extended period and are then provided parentral nutrition, displacing electrolytes and causing other metabolic complications.

A 28-year-old male presents with complaints of pain along the cervical chain of lymph nodes after ingesting alcohol. In further discussion with him, he mentions having occasional drenching night sweats that have been occurring more frequently over the last month. He has also had a low-grade temperature of 38C on and off over the last several months, but attributed both to the summer weather. He also has lost weight over the last six months without trying. Recently, he has noted a generalized pruritus as well. He has palpable cervical chain lymph nodes. Histologic examination of a lymph node will most likely show which of the following?

A Auer rods
B IgG lyte chains
C Philadelphia chromosome
D Reed-Sternberg cells
E VZV inclusion bodies

Reed-sternberg cells
This patient is presenting with findings consistent with Hodgkin Lymphoma. Pain in involved lymph nodes immediately after the ingestion of alcohol is nearly specific to this disease. On histologic examination of lymph tissue, Reed-Sternberg cells can be seen. Auer rods are seen in AML. Philadelphia chromosome is typically seen in CML. IgG lyte chains are seen in multiple myeloma. VZV inclusion bodies would not be seen in this setting.

A 1-year-old boy is brought to the emergency department by his parents, who state that the child refuses to walk or crawl and begins crying when they stand him. Swelling to his right knee is noted; it is also warm to the touch and pain response is noted. His parents state that it seemed to start a couple of days ago and has gotten worse. They don't recall a trauma, but state that he seems to bruise easily. The child's mother states that she also bruises easily. Vitals are as follows: Temp: 38.0°C, HR: 70, RR: 15. What laboratory finding would you expect?

A Prolonged aPTT (activated partial thromboplastin time)
B Prolonged bleeding time
C Prolonged PT (prothrombin time)
D Prolonged thrombin clotting time
E Thrombocytopenia

This patient has hemophilia A. Patients with severe hemophilia A have a prolonged aPTT; all of the other tests should be within the normal range.

A 59-year-old female patient with no complaints is undergoing routine physical examination in your family practice office and has a hypochromic, microcytic anemia that was not present on her physical examination last year. Which of the following is the most important cause to rule out in this patient?

A acute pathology secondary to aplastic anemia
B chronic anemia secondary to colon cancer
C Falconi's anemia
D iron deficiency anemia
E medication-induced anemia

chronic anemia secondary to colon cancer
While there are many causes of hypochromic, microcytic anemia, high on the differential is blood loss, commonly occult and long standing in nature. This can be from heavy menstruation or gastrointestinal loss. This later is commonly caused by an occult colon cancer. This patient should initially receive a sensitive screen such as an immunochemical fecal occult blood test (IFOBT) and, given her age, a colonoscopy should be strongly considered.

During a pre-surgical workup, a patient reports that she had received Desmopressin following the birth of her son. Her CBC is within normal range, as well as her PT and aPTT. What is the most likely cause of her needing this medication?

A Allo immunization following vaginal delivery
B Hemolytic anemia
C Iron deficiency anemia
D Thalassemia
E Von Willebrand's disease

Von Willebrand's disease
Desmopressin is the mainstay of therapy for people with von Willebrand's disease. Hemolytic anemia, iron deficiency anemia, and thalassemia would all have abnormal findings on the CBC. Allo immunization does not require desmopressin therapy.

A 29-year-old female complains of a two-month history of easy bruising. She describes that the bruising is located primarily on her shins, but has noted them on other areas as well. She also describes red freckles on her lower extremities. On exam, you note non-blanching and non-palpable purpura to both legs and petechiae. She denies recent illnesses and states that she has essentially been feeling fine. The PE is normal other than the skin findings. Which diagnostic study would be most helpful as a first choice?

A Antiplatelet antibody test
B Complete blood count
C Direct antiglobulin test
D Reticulocyte count
E Thyroid function

CBC
This patient is exhibiting signs of Idiopathic (Immune) thrombocytopenic purpura. The diagnosis is based on history, physical examination, blood count, and blood film. The American Society of Hematology guidelines recommend no further diagnostic studies. The other studies may be useful if you believe another underlying disease is causing the symptoms and thrombocytopenia, but a complete blood count should be sufficient.

A 28-year-old male presents with a diagnosis of Hemophilia A. Which of the following would he be deficient in?

A Christmas factor
B Factor VII
C Factor VIII
D Factor IX
E Von Wildebrand's factor

Hemophilia A is caused by a defective synthesis of Factor VIII.
Factor IX and Christmas factor are deficiencies in Hemophilia B. Deficiency of factor VII can exacerbate bleeding issues, but are not typically seen in Hemophilia A. Von Willebrand's factor is a loss of this platelet-binding multimer, and most patients will have enough factor VIII but not the ability to bind factor VIII. In very severe cases of vWF, factor VIII may become low enough to produce symptoms similar to those found in factor VIII deficiency.

A 62-year-old female presents to your office with a complaint of hematuria. She had a cardiac valve replacement three months ago and was placed on warfarin. You perform an in-office international normalized ratio (INR) and discover it is 6.8. Which of the following agents would provide the appropriate reversal of the effects of the warfarin?

A folic acid
B Fresh frozen plasma
C vitamin B
D vitamin K (phytonadione)
E protamine sulfate

vit k
Excessive anticoagulant effect and bleeding from warfarin can be reversed by stopping the drug and administering oral or parenteral vitamin K1 (phytonadione). The speed and extent of reversal must be balanced against the risk of recurrent thromboembolism in patients who require therapeutic anticoagulation. For example, an over-anticoagulated patient with a prosthetic mitral valve may develop fatal thrombosis if supratherapeutic anticoagulation is rapidly and fully reversed.

A 29-year-old female complains of a two-month history of easy bruising. She describes that the bruising is located primarily on her shins, but has noted them on other areas as well. She also describes red freckles on her lower extremities. On exam, you note non-blanching and non-palpable purpura to both legs and petechiae. She denies recent illnesses and states that she has essentially been feeling fine. The PE is normal other than the skin findings. A platelet count of 118,000 is noted. What is the most likely diagnosis?

A Antiphospholipid syndrome
B Idiopathic thrombocytopenic purpura
C Systemic lupus eruthematosus
D Thrombotic thrombocytopenic purpura
E Von Willebrand's disease

ITP
Diagnosis is based on clinical manifestations including history, physical exam, blood count, and blood film. Typically, these patients have felt fine until they notice development of purpura and petechiae. No additional physical findings are typically found. Blood counts note a thrombocytopenia, with other tests usually in the normal range.

A 29-year-old male who appears healthy was injured playing basketball and received an x-ray of his spinal column. A mass was noted on the film and referred to the hematologist. He denies shortness of breath or chest pain. He does note drenching night sweats that he has had for several weeks, but assumed it had to do with the hot weather. On exam he has painless lymphadenopathy and is underweight for his height. A CBC is normal and LDH is elevated. What is the most likely diagnosis?

A Acute myelogenous leukemia
B Cat scratch disease
C Infectious mononucleosis
D Non-Hodgkin lymphoma
E Sarcoidosis

Non-Hodgkin lymphoma
This patient presents with classic signs of Non-Hodgkin lymphoma, consisting of mediastinal mass, "B" symptoms, and painless lymphadenopathy. AML would not have a normal CBC. Cat scratch disease would have painful lymphadenopathy if present. Infectious mononucleosis would have lymphadenopathy and fever, but not drenching night sweats or mediastinal mass. Sarcoidosis would have granulomatous disease noted on a chest x-ray, not a large mediastinal mass.

A known patient who carries the diagnosis of hemophilia A wants to know what medications they can use to relieve the symptoms of the flu. Which of the following is the most appropriate to recommend?

A Acetaminophen
B Acetylsalicylic Acid
C Diclofenac
D Ibuprofen
E Naproxen

Acetaminophen
Acetaminophen is the only product on the list that won't exacerbate or interfere with platelet aggregation. All other products are to be avoided, as they interfere with platelet aggregation and exacerbate bleeding.

A 24-year-old female patient develops her second deep vein thrombosis (DVT) in two years. After a hypercoaguability work up, you discover that she has a factor V Leiden abnormality. How long should she remain on oral anticoagulation therapy?

A She does not need anticoagulation therapy.
B You may stop her anticoagulation therapy when her current DVT has resolved.
C You may stop her anticoagulation therapy in six months.
D You may stop her anticoagulation therapy in one year.
E She needs lifetime anticoagulation therapy.

She needs lifetime anticoagulation therapy.
When patients have a deep vein thrombosis, it usually warrants a hypercoaguability work up to rule out genetic causes. While complicated and sometimes requiring hematology consultation, patients with hypercoaguable genetic disorders such as a factor V Leiden abnormality generally require lifetime anticoagulation.

A 32-year-old female is brought into the emergency department by her partner. His report indicates that she had been in her usual state of good health until a couple of days ago. At that time she started to complain of feeling fatigued. She now appears jaundiced and lethargic, and is complaining of chest pain. On exam, her spleen is palpable. Hemoglobin is 6 g/dl and she is Coombs positive. What is the most likely diagnosis?

A Autoimmune hemolytic anemia
B Glucose-6-phosphate dehydrogenase deficiency
C Hereditary spherocytosis
D Pyruvate kinase deficiency
E Thalassemia

Autoimmune hemolytic anemia
The onset of autoimmune hemolytic anemia (AHA) is often abrupt and dramatic. Anemia can develop in days, along with jaundice and splenic enlargement. When this triad is present, the suspicion for AHA must be high. The diagnostic test for AHA is the Coombs test. If positive, it confirms the presence of the antibody on the red cells. All other diseases listed are Coombs negative hemolytic anemias.

This common malignancy is diagnosed in patients younger than 15 years of age, has an incidence peak during early childhood (2 to 4 years old), and is seen more prominently in industrialized nations. What is the name of this malignancy?

A Acute Lymphoblastic Leukemia
B Acute Myelogenous Leukemia
C Chronic Lymphocytic Leukemia
D Chronic Myelogenous Leukemia
E Lymphoma

ALL
Acute Lymphoblastic Leukemia is the most common malignancy diagnosed in patients younger than age 15, and has a peak incidence of 2 to 4 years of age. It is seen more often in males, and the pattern affected suggests a leukemogenic contribution from factors associated with industrialization.

A 42-year-old African American male is admitted to the hospital with heme positive urine and anemia. He recently completed a course of trimethoprim/sulfamethoxazole for a urinary tract infection. What is the most likely cause of these findings?

A Glucose-6-phophate dehydrogenase deficiency
B Factor VIII disorder
C Idiopathic thrombocytopenic purpura
D Sickle cell anemia
E Thalassemia

Glucose-6-phophate dehydrogenase deficiency
G6PD deficiency is an X-linked recessive disorder affecting 10 to 15% of African American males. Hemolysis occurs as a result of oxidative stress on the red cells after exposure to infection or certain drugs, and leads to a hemolytic episode. Common drugs include sulfonamides. Factor VIII and ITP are coagulation disorders, not anemias. Sickle cell and thalassemia are not transient and do not usually present this late in life.

Which of the following is a potential adverse effect associated with unfractionated heparin (UFH)?

A hyperglycemia
B hypothyroidism
C thrombocytopenia
D excessive cough
E muscle cramps

thrombocytopenia
Heparin-induced thrombocytopenia (HIT) is a potentially serious complication of unfractionated heparin therapy, usually occurring within 4 to 10 days after heparin treatment has started. Fortunately, current estimates show that it is infrequent, occurring in approximately 0.3% to 3.0% of patients receiving UFH for more than 4 days. HIT should immediately be suspected in a patient who develops deep vein thrombosis or pulmonary embolism while receiving UFH.

A 4-year-old is diagnosed with severe thrombocytopenia, causing spontaneous bleeding two weeks after having influenza. The child has no evidence of anemia, neutropenia, or anything else that raises a suspicion for an alternate diagnosis, and there are no atypical findings on the blood film. What is the most likely diagnosis?

A Autoimmune hemolytic anemia
B Immune thrombocytopenic purpura
C Thalassemia
D Thrombotic thrombocytopenic purpura
E Von Willebrand's disease

Immune thrombocytopenic purpura
In children with no other potential causes, the most likely diagnosis is immune thrombocytopenic purpura. The disease must be distinguished from acute lymphoblastic leukemia in this population prior to finalizing the diagnosis. Autoimmune hemolytic anemia would present with an anemia. Thalassemia is an inherited hematologic disorder, von Willebrand is a bleeding disorder typically with normal platelet counts, and thrombotic thrombocytopenic purpura typically has an associated anemia and a lack of bleeding.

A 9-year-old male presents with an acute onset of petechiae, ecchymoses, and gingival bleeding. He has pallor, fatigue, and bony pain. A pancytopenia is noted on CBC. Ebstein-Barr is negative. Lymphoblasts are noted on smear. Vitals reveal a temperature of 100.8F, HR 74, and RR 20. A few shoddy cervical nodes are noted. What is the most likely diagnosis?

A Acute lymphoblastic leukemia
B Aplastic anemia
C Infectious lymphocytosis
D Infectious mononucleosis
E Lymphoma

ALL
This patient has acute lymphoblastic leukemia (ALL). While all the diseases would be in the differential, only ALL fits all the findings. Infectious mononucleosis is excluded with a negative EBV. Infectious lymphocytosis would not have a pancytopenia or increased blasts on smear. Aplastic anemia would have a pancytopenia but not bony pain. Lymphoma typically does not have a pancytopenia associated with the disease.

A 67-year-old male presents for his annual physical in February. On examination, you note nontender cervical and axillary adenopathy. He states that he has felt those lumps for several weeks, has noted feeling more fatigued, and has decided that he had a cold. On further inspection, you note a mildly enlarged spleen, and the patient states that he hasn't been eating as much as he usually does. A CBC reveals an elevated lymphocyte count. These findings are most consistent with which diagnosis?

A Acute infection lymphocytosis
B Chronic lymphocytic leukemia
C Rickettsiosis
D Stress lymphocytosis
E Systemic lupus erythematosus

CLL
The findings in this patient are consistent with chronic lymphocytic leukemia. Rickettsiosis and systemic lupus erythematosus tend to cause a lymphocytopenia rather than lymphocytosis. Acute infection lymphocytosis do not have either lymph node enlargement or splenomegaly, and stress lymphocytosis is short lived following trauma, surgery, or other insults to the body.

Upon examination of a 24-year-old male, you note painless lymphadenopathy along the cervical and femoral chains. He notes that he has had to change his bedding daily because he soaks his sheets from sweat. He has also lost 35 pounds without trying to over the last three months. He thinks that he has had fevers at night but has never checked. This is a classic description of which of the following?

A "B" symptoms
B Cat scratch fever
C Neutropenia
D Sickle cell crisis
E Sideroblastic anemia

"B" symptoms
This patient is presenting with signs and symptoms of non-Hodgkin's lymphoma. Many patients with lymphoma exhibit fever, drenching night sweats, and weight loss—these symptoms are referred to as "B" symptoms. Cat scratch fever usually has painful lymphadenopathy. Sickle cell crisis and sideroblastic anemia do not produce non-painful lymphadenopathy. Neutropenia involves a low white cell count.

A 51-year-old female undergoing chemotherapy for stage IIC breast cancer presents to her primary care provider with a complaint of fatigue, becoming tired very easily, and an inability to perform any of her normal daily activities. What is the most common reversible cause of chemotherapeutic -related fatigue?

A anemia
B anxiety
C chronic pain
D neutropenia with sepsis
E sleep disturbance

Anemia
Fatigue is one of the most common symptoms in cancer patients, experienced by 70% to 100% of those receiving cancer treatment. It is most commonly related to the chemotherapeutic agent itself and will resolve when treatment is completed. In the interim, anemia is the most common cause and this can be treated with hematopoietic growth factors, erythropoietin (epoetin alfa and epoetin beta), granulocyte colony-stimulating factor (G-CSF), granulocyte-macrophage colony-stimulating factor (GM-CSF).

A 68-year-old female presents with symptoms of kidney failure. Her creatinine is elevated at 1.9, and monoclonal light chains are seen in the urine. She complains of fatigue and bony pain that has been increasing over the last several months. This patient is most likely experiencing symptoms from which disease?

A Acute myelogenous leukemia
B Chronic myelogenous leukemia
C Hodgkin lymphoma
D Multiple myeloma
E Non-Hodgkin lymphoma

Multiple Myeloma
30 to 50% of patients presenting with multiple myeloma will have some form of renal impairment. The most frequent cause is the formation of myeloma cast nephropathy. This is due to formation of tubular casts in the distal nephron, formed by the binding of light chains to uromodulin. These tubular casts obstruct the distal nephron and parts of the ascending loop of Henle, and contribute to development of interstitial nephritis. Light chains are not seen in the urine of any of the other diseases listed.

What absolute tissue pressure generally is used as a guideline for diagnosing compartment syndrome?

A 10 mm Hg
B 20 mm Hg
C 30 mm Hg
D 40 mm Hg
E 50 mm Hg

30 mmHg
Many trauma surgery services use an absolute tissue pressure of approximately 30 mm Hg as the threshold for diagnosing compartment syndrome. Based on the entire clinical picture, patients with numbers in that range or higher will likely require surgical decompression with a fasciotomy, while lower numbers will probably be managed with a more conservative approach.

Which of the following signs and symptoms is associated with the abdominal pain secondary to chronic intestinal ischemia?

A Guarding and rigidity
B Fear of eating
C Nausea and vomiting
D Bloody diarrhea
E Positive obturator and psoas signs

Fear of eating
The clinical symptoms associated with chronic intestinal ischemia include severe epigastric pain following meals, which results in weight loss and fear of eating. Nausea, bloody diarrhea, and vomiting as well as guarding and rigidity are consistent with acute intestinal ischemia. Obturator and psoas signs are indicative of acute appendicitis.

Which of the following conditions is the most common cause of massive lower gastrointestinal bleeding?

A Hemorrhoids
B Colon cancer
C Diverticular disease
D Upper gastrointestinal hemorrhage
E Meckel diverticulum

Diverticular disease
Although hemorrhoids are a common cause of lower gastrointestinal hemorrhage, they do not cause massive hemorrhage. Massive lower gastrointestinal hemorrhage is most commonly due to diverticular disease. Upper gastrointestinal hemorrhage may present as massive lower hemorrhage due to the cathartic effect of blood. Colon cancer usually presents with occult bleeding. Meckel diverticulum may also present significant lower gastrointestinal hemorrhage, but it affects only approximately 2% of the population and is therefore not as common

Which of the following etiologies is the most common cause of nontraumatic subarachnoid hemorrhage (SAH)?

A Cerebral aneurysm
B Poorly controlled hypertension
C Anticoagulant use
D Arteriovenous malformation

cerebral aneurysm
Congenital cerebral aneurysms or Berry aneurysms account for 75% to 80% of nontraumatic subarachnoid hemorrhages (SAHs). Poorly controlled hypertension and anticoagulant use are more commonly associated with intracerebral hemorrhages (ICH). AVMs can cause either SAH or ICH.

A 67-year-old female with a history of oxygen dependent emphysema presents with a 4-hour history of increasing shortness of breath and pleuritic chest pain on the right side. Her resting oxygen saturation rate is 90%, and she is having pain on inspiration. On examination, the patient has decreased lung sounds with wheeze on the left and absent sounds on the right. There is also tympany to percussion on the right. Based on these findings, what is the best therapy for this patient?

A Needle insertion to right chest wall
B Supportive care
C Increased oxygen delivery
D Chest tube insertion
E Nebulized albuterol

chest tube insertion
The treatment for this patient, who has a pneumothorax, is chest tube insertion and reinflation of the lung. Once the air leak has been eliminated and the lung appears reinflated on serial chest x-rays, the chest tube may be removed.

A 1 day-old boy develops progressing abdominal distension, bilious vomiting and failure to pass a meconium stool. Abdominal radiographs show dilated loops of small bowel. Which of the following is the most likely diagnosis in this patient?

A Cystic Fibrosis
B Hypothyroidism
C Imperforate anus
D Intussusception
E Pyloric Stenosis

Cystic Fibrosis
A The patient presents with a meconium ileus consistent with a diagnosis of cystic fibrosis (A). Pyloric stenosis (E) typically presents between 3 and 6 months of age, while intussusception (D) presents later (6 to 24 months). Imperforate anus (C) presents at birth, but infants typically lack acute abdominal distention and bilious vomit. Other common causes of intestinal obstruction in a newborn include meconium plug syndrome, Hirschsprung Disease, Intestinal Atresia, and Midgut Volvulus.

A 74-year-old woman presented with the new onset of seizures. An MRI with gadolinium showed a parasagittal mass with homogenous enhancement and a "dural tail." What is the appropriate management of this patient?

A Radiation therapy
B Chemotherapy
C Surgical resection
D Surgical resection with chemotherapy

Surgical resection
The clinical presentation is consistent with a meningioma. Meningiomas are commonly located in the parasagittal region, the convexity of the brain, sphenoid ridge, or posterior fossa. Radiographic features on MRI include homogenous enhancement and evidence of a "dural tail" indicating the origin of the tumor. Since meningiomas are a benign tumor, the primary treatment is surgical removal. In the event of a subtotal resection or if the meningioma is found to be malignant, surgical resection is followed by radiation therapy.

A 56-year-old man is diagnosed with a carcinoma of the sigmoid colon by colonoscopy. Which of the following tests should be performed as part of the preoperative evaluation for distant metastasis?

A Carcinoembryonic antigen level
B CT of the chest
C Endorectal ultrasound
D CT scan of the abdomen
E Bone scan

Ct of the abdomen
Abdominal/pelvic CT scans and chest radiographs should be obtained as part of the preoperative staging of colon carcinoma for the evaluation of distant metastasis. CT scan of the chest is indicated only if the chest radiograph is abnormal. Endorectal ultrasound is useful in further staging rectal carcinoma. Bone scan is not indicated as a routine diagnostic study in the preoperative staging of colon cancer. Although carcinoembryonic antigen levels are performed preoperatively, they are not helpful in staging and are useful only in the postoperative follow-up of patients to monitor for a recurrence.

A patient is recovering from having a total thyroidectomy two days ago for medullary thyroid cancer. An extensive neck dissection was required during the surgery. Post-operative lab testing reveals a low serum calcium level. Which of the following clinical presentations will most likely occur in this patient?

A Constipation
B Anorexia
C Polyuria
D Bone pain
E Paresthesias

Paresthesias
The correct choice is E, paresthesias. Circumoral paresthesias are signs of hypocalcemia. Hypocalcemia can occur after any type of neck surgery that may have resulted in destruction of the parathyroid glands. Choices A through D are symptoms of hypercalcemia and may be seen in hyperparathyroidism.

A 88-year-old female is found lying on the ground in a supine position after sustaining a fall in her house from tripping on the rug.. Her leg is shortened, abducted and externally rotated, and she is complaining of pain to the right leg with radiation to the knee. Based on the history of the patient, what type of injury has this patient sustained?

A Non-displaced pelvic fracture
B Hip dislocation
C Femoral shaft fracture
D Femoral neck fracture
E Distal femur fracture

Femoral Neck Fracture
When a fracture occurs in the femoral neck that includes displacement, the leg appears shortened, abducted and externally rotated. The tension that exists from muscles attached above and below the fracture site results in shortening of leg length after the fracture as the muscles contract. If there is no displacement of the bone, no leg length changes should occur. Generally, hip fractures would not result in a lengthening of the leg, so all answers that include this option would be wrong. When the leg appears shortened, flexed, adducted, and internally rotated, a posterior hip dislocation should be suspected. A leg that is flexed, abducted and externally rotated suggests an anterior hip dislocation. Posterior hip dislocations are far more common than anterior dislocations, accounting for more than 90% of all hip dislocations.

A 70-year-old man with a history of hypertension, DM Type 2, and hyperlipidemia is seen for pre-operative evaluation prior to left knee replacement. On auscultation, a very soft high-frequency decrescendo early diastolic murmur is heard at the upper left sternal border. Utilizing isometric hand grip exercises, the murmur increases in intensity and can be heard radiating to the left sternal border and apex. Given the patient's physical exam findings, which of the following is the most likely diagnosis?

A Aortic stenosis
B Ventricular septal defect
C Mitral stenosis
D Aortic regurgitation/insufficiency
E Mitral regurgitation/insufficiency

Aortic regurgitation
Isometric hand grip exercises will increase the intensity of the murmur of aortic regurgitation, which is usually described as a high-frequency decrescendo early diastolic murmur that is heard best at the left upper sternal border or at the right upper sternal border. Radiation, if it occurs, is frequently to the lower left sternal border and the apex. Isometric hand exercises increase arterial and left ventricular pressure, which increases the flow across the aortic valve, thereby increasing the murmur's intensity.

Which type(s) of Salter-Harris fractures can generally be treated with closed reduction and cast immobilization?

A Type I
B Types I and II
C Types I, II, and III
D Types I, II, III, and IV
E Types I, II, III, IV, and V

Types 1, 2, and 3
Minimally displaced Salter-Harris types I, II, and III fractures generally can be treated with immobilization only. Types IV and V involve the cartilage of both the articular surface and the growth plate. To ensure proper alignment and a congruous joint surfaces open reduction and internal fixation is usually necessary.

You are evaluating a patient who is having decreased sensation to his arm after he sustained a head on injury while playing football. He is stating that his right arm has decreased sensation that goes into the hand, but he is slightly vague on being descriptive. As you perform the physical examination, what part of the body would exhibit sensation for the C7 component of the brachial plexus?

A Lateral forearm
B Lateral upper arm
C Medial forearm
D Medial upper arm
E Third Finger

Third finger
The sensory nerve emanating from the C7 level is responsible for sensation of the third finger. The lateral forearm is supplied by C6. The lateral upper arm is supplied by C5. The medial upper arm is supplied T1 and the medial forearm is supplied by C8.

A 5-year-old male is being evaluated for an acute injury to the right ankle. On the x-ray of the ankle there is a distal tibia fracture that involves the separation of the epiphysis, as well as a small non-displaced chip fracture of the metaphysis of the tibia. Based on these findings, what type of Salter-Harris fracture does this child have?

A I
B II
C III
D IV
E V

II
The growth plate is the most fragile part of the bone prior to bone maturation and thus is usually the first structure disrupted when force is applied. Statistically, Type II fractures are most common - those that involve both the growth plate and a chip fracture of the metaphysis.

A 56-year-old insulin dependent diabetic has been under your evaluation for his diabetes for several years. The patient has a 3-year history of diabetic neuropathy to the right foot, and may have suffered an injury to the foot without knowing due to loss of sensation. The patient now presents with a tender, reddened, and swollen right foot for the last 10 days that is also warm to the touch. You suspect that this patient may have an acute case of osteomyelitis. Based on this history, what bacterial organism is most commonly the cause of osteomyelitis?

A Group A beta-hemolytic streptococci
B Hemophilus influenzae
C Mycoplasma
D Pseudomonas aeruginosa
E Staphylococcus aureus

Staph aureus
Osteomyelitis is an infection in a bone and can occur in patients of all ages. The most common organism implicated in osteomyelitis across all age groups is Staphylococcus aureus. It can enter the bone through multiple mechanisms including by direct inoculation during an open fracture or during surgical intervention following a fracture (most common mechanisms for adults) or by hematogenous spread from another source (the usual cause in children). Hemophilus influenzae was a much more common organism in pediatric osteomyelitis in the past, but its prevalence is decreasing due to routine immunizations. Group A beta-hemolytic streptococci is the second most common organism found in osteomyelitis in children, while Pseudomonas aeruginosa is the second most common pathogen in adults. Mycoplasma induced osteomyelitis is relatively rare and usually confined to immunocompromised patients.

What nerve is most commonly injured in a mid- or distal humeral shaft fracture?

A Axillary
B Median
C Peroneal
D Radial
E Ulnar

Radial Nerve
Because of the radial nerves proximity to the humerus, mid and distal shaft fractures with significant displacement can cause a radial nerve injury. Median and ulnar injuries are more commonly associated with forearm injuries. Axillary nerve injuries are most common in anterior shoulder dislocations and peroneal nerve damage occurs as a result of lower leg insult. (Tuncali, et. al., "Upper Extremity Nerve Injuries: The Significance of Soft Tissue Associations,"

A 70-year-old man with a history of hypertension, DM Type 2, and hyperlipidemia is seen for preoperative evaluation prior to left knee replacement. On auscultation, a very soft high-frequency decrescendo early diastolic murmur is heard at the upper left sternal border. Which of the following maneuvers would be the most appropriate to choose to increase the intensity of the murmur for better identification?

A Isometric hand grip exercise
B Listening with the bell at the apex with the patient in the left lateral decubitus position
C Inspiration, followed by the patient holding his/her breath
D The Valsalva maneuver
E Having the patient lie flat with the knees bent

Isometric hand grip exercise
Isometric hand grip exercises will increase the intensity of the murmur of aortic regurgitation, which is usually described as a high-frequency decrescendo early diastolic murmur heard best at the left upper sternal border or at the right upper sternal border. Radiation, if it occurs, is frequently to the lower left sternal border and the apex. Isometric hand exercises increase arterial and left ventricular pressure, which increases the flow across the aortic valve, thereby increasing the murmur's intensity. Choice B is best used when listening to the murmur of mitral stenosis. Choice C will increase the AP diameter, making it more difficult to hear the murmur. Choice D, the Valsalva maneuver, will decrease the intensity of the murmur. Choice E is the best position for the abdominal exam, especially in males.

A 63-year-old white male is seen in the ambulatory outpatient clinic with complaints of midepigastric pain, weight loss, and jaundice. On examination, he is jaundiced and his sclerae are icteric. On palpation of the abdomen, you find a distended nontender gallbladder. Which of the following is the most likely diagnosis?

A Gastric carcinoma
B Chronic pancreatitis
C Pancreatic carcinoma
D Choledocholithiasis

Pancreatic carcinoma
Pancreatic carcinoma presents with weight loss, jaundice, and midepigastric pain. A palpable, nontender gallbladder (Courvoisier sign) is more often associated with a pancreatic malignancy than cholelithiasis, especially if the tumor is in the head of the pancreas. In acute cholecystitis, the obstruction in the cystic duct is associated with inflammation, resulting in a tender gallbladder on palpation of the right upper quadrant (Murphy sign); obstruction of the common bile duct in choledocholithiasis will result in jaundice but not weight loss. Gastric carcinoma will present with midepigastric pain and weight loss but not jaundice or a palpable gallbladder. Midepigastric pain is the most common symptom seen in chronic pancreatitis, and weight loss may be seen in association with malabsorption secondary to exocrine insufficiency.

A 66-year-old woman presents to the emergency department with a complaint of abdominal pain and distension for the past 3 days. Examination reveals a protuberant abdomen with diminished bowel sounds and tympany to percussion. Flat and upright abdominal radiographs reveal distended loops of bowel with prominent haustral markings. Which of the following etiologies is the most likely cause of the patient's condition?

A Volvulus
B Adenocarcinoma
C Diverticular disease
D Strangulated hernia
E Adhesions

Adenocarcinoma
Large bowel obstructions are most commonly caused by an adenocarcinoma (65%). This is followed in decreasing incidence by diverticular scarring and volvulus. Adhesions are the most common cause of small-bowel obstruction but are rare as a cause of large bowel obstruction. The presence of haustral markings on radiographic evaluation helps differentiate between small and large bowel involvement.

In a boxer's fracture, the presence of how much angulation of the fifth metacarpal neck would require referral for reduction as opposed to treatment with a simple ulnar gutter splint?

A Less than 10 degrees
B 10-19 degrees
C 20-29 degrees
D 30-39 degrees
E Greater than 40 degrees

Greater than 40 degrees
When there is greater than 40 degrees of angulation or an extensor lag (the patient cannot fully extend the affected finger) then a referral is required. Lesser degrees of angulation without an extensor lag can generally be handled conservatively with an ulnar gutter.

A patient who is an Ashkenazi Jew and is homozygous for Factor XI deficiency requires abdominal surgery. Which hemostatic laboratory testing outcomes would you expect to find?

A Decreased platelets, normal aPTT, prolonged PT
B Decreased platelets, prolonged aPTT, normal PT
C Normal platelets, normal aPTT, prolonged PT
D Normal platelets, prolonged aPTT, normal PT
E Normal platelets, prolonged aPTT, prolonged PT

D Normal platelets, prolonged aPTT, normal PT
Patients with factor XI deficiency have a prolonged aPTT, normal PT, and normal platelets count.

A 54-year-old man presents to the emergency department with crampy abdominal pain, nausea, and vomiting. The patient has not passed gas or had a bowel movement for at least 10 hours. On examination, the abdomen is distended and there are high-pitched bowel sounds with rushes. A plain radiograph of the abdomen reveals cecal distension to 12 cm. What is the most appropriate definitive management for this patient?

A Intravenous fluids
B Nasogastric suction
C Observation
D Surgical exploration

Surgical exploration
Massive distention of the cecum, as detected on plain radiograph, is typically seen in "closed loop" obstructions where the ileocecal valve is competent. When distention approaches 12 cm, there is an increased risk of perforation and/or gangrene. Expedient surgical intervention is indicated. Although observation with intravenous fluids and nasogastric decompression are important adjuncts to management, surgical exploration is the only way to rapidly address this emergent situation.

A 57-year-old woman underwent a hemicolectomy for adenocarcinoma of the colon. Which of the following recommendations is part of postoperative monitoring for a potential recurrence?

A Annual fecal occult blood testing
B Annual chest radiograph
C Annual CA 19-9 testing
D Annual flexible sigmoidoscopy
E Annual colonoscopy

Annual colonoscopy
Routine follow-up after surgical resection of a colon cancer includes annual colonoscopy not sigmoidoscopy, which only assesses the distal colon. The tumor marker for colon cancer is carcinoembryonic antigen (CEA) not carbohydrate antigen 19-9 (CA 19-9), which is used for pancreatic cancer. There is no role for annual chest films or fecal occult blood testing to monitor for a recurrence.

Following emergent appendectomy, a 58-year-old obese male develops a temperature of 102.4˚F, 18 hours postoperatively. His respiratory rate is 26 and his pulse is 116bpm. A physical exam reveals scattered fine rales. What is the most likely diagnosis?

A Atelectasis
B Aspiration pneumonitis
C Pleural effusion
D Pneumonia
E Pulmonary embolus

Atelectasis
Pulmonary alveoli collapse, also known as atelectasis, occurs during operative procedures for a variety of reasons, including decreased clearance of secretions and decreased intra-alveolar pressure. Postoperatively, often due to pain, patients may not mobilize secretions appropriately, also contributing to atelectasis. Atelectasis is the most common postoperative pulmonary complication, and is often associated with emergent and prolonged surgeries, especially those of the thorax and abdomen. Atelectasis is associated with fever, an increased respiratory rate, an increased pulse, and lung exam findings ranging from normal to rales and decreased breath sounds. Symptoms usually present within the first 48 hours postoperatively. Pulmonary aspiration pneumonitis, although possible, is less likely due to appropriate preoperative and intraoperative measures being utilized to decrease risk. Pneumonia is also a common postoperative complication, due to the same contributing factors as atelectasis. Physical exam findings may also be similar. However, postoperative pneumonia is likely to become evident between 24 and 96 hours postoperatively.

A 54-year-old man complains of persistent midepigastric abdominal pain 2 weeks following the diagnosis of acute pancreatitis. The patient also complains of anorexia but no fever or chills. There is a palpable mass in the midepigastrium; bowel sounds are normal in all four quadrants. Which of the following is most likely diagnosis?

A Adynamic ileus
B Pancreatic carcinoma
C Infected pancreatic necrosis
D Pancreatic pseudocyst

Pancreatic pseudocyst
Pancreatic pseudocysts are the most common complication associated with acute pancreatitis. A pseudocyst should be suspected for a patient who has continued abdominal pain, the development of an abdominal mass, and continued elevations of amylase or lipase levels following an episode of acute pancreatitis. An adynamic ileus would be associated with abdominal distension and changes in bowel sounds; an infected area of necrosis within the pancreatic gland would be associated with fever. Pancreatic cancer may be seen in conjunction with chronic pancreatitis.

A 45-year-old man presents to the office with the complaint of perianal pain and bleeding. Examination reveals an anal/perianal mass complex. Biopsy is taken and the results are positive for epidermoid carcinoma of the anus. Which of the following treatment options would be the most appropriate therapy for this patient?

A Local resection, chemotherapy, and external beam radiation
B Abdominoperineal resection
C Chemotherapy only
D Local resection only

Local resection, chemotherapy, and external beam radiation
Epidermoid carcinoma of the anal canal is a slow-growing tumor that often presents as an anal or perianal mass. Wide excision, followed by 5-fluorouracil, mitomycin, and external beam radiation (Nigro Protocol), typically results in a greater than 80% cure rate. The presence of inguinal lymph node metastasis is a poor prognostic indicator. Management of recurrences is typically achieved by performing an abdominoperineal resection

A 58-year-old man with a 20-year history of gastroesophageal reflux disease (GERD) presents with progressive dysphagia for 5 months associated with a 20-lb weight loss. ). Which of the following is the most likely diagnosis?

A Achalasia
B Esophageal leiomyoma
C Uncomplicated reflux esophagitis
D Esophageal carcinoma

Esophageal carcinoma
Dysphagia on a background of GERD is an alarm signal for cancer, since GERD is related to increased risk for esophageal adenocarcinoma. Esophageal cancer is associated with a progressive course of dysphagia, first to bulky foods, then softer foods, and then liquids as the tumor invades the esophagus; significant weight loss is almost universal at the time of presentation. Barium swallow demonstrates narrowing at the tumor site with normal appearance of the remainder of the esophagus. Achalasia is a motor disorder characterized by dysphagia to both liquids and solids as well as regurgitation of food. Patients with achalasia typically drink large amounts of liquids to force their food down and have problems with aspiration pneumonia. Barium swallow in achalasia typically shows a dilated esophagus with a narrowing at the lower esophageal sphincter (bird's beak). Leiomyomas are generally asymptomatic. Patients with reflux esophagitis will complain of epigastric or substernal pain that is worse when supine or leaning forward

A patient is scheduled for a mitral valve replacement. Which of the following pharmacologic agents would be recommended for surgical prophylaxis? There are no known drug allergies.

A Cefazolin
B Vancomycin
C Ciprofloxacin
D Nafcillin

Cefazolin
Cefazolin is used as prophylaxis for the majority of clean surgical procedures. For cases in which there is an increased likelihood of encountering gram-negative organisms or anaerobic bacteria, a second-generation cephalosporin is recommended to provide broader coverage. Vancomycin is an alternative if the patient has an allergy to cephalosporin antibiotics.

A 58-year-old man is in the hospital postoperative day 3 after a laparoscopic right colon resection. Your morning labs reveal a serum potassium level of 2.9 mEq/L (normal 3.5-5.0 mEq/L) despite aggressive potassium replacement during the previous shift. At this time you should check which of the following laboratory values?

A Magnesium
B Calcium
C Phosphorous
D Sodium
E Albumin

Magnesium
Hypokalemia is a common electrolyte disturbance in surgical patients. It can be caused by enhanced losses, hyperaldosteronism, inappropriate replacement, and intracellular shifts caused by alkalosis. Symptoms of hypokalemia may include constipation, neuromuscular weakness, diminished tendon reflexes, paralysis, and distinctive electrocardiographic changes. Concomitant deficiencies in magnesium can contribute significantly to the development of hypokalemia as well as hypocalcemia. In the surgical patient with persistent hypokalemia refractory to potassium administration, one should check magnesium levels and correct as appropriate.

Approximately what percentage of patients with a solid primary tumor elsewhere will end up with metastatic disease of the vertebrae during the clinical course of their cancer?

A 10%
B 20%
C 30%
D 40%
E 50%

50%
Fifty percent of cancer patients will develop metastatic disease of the vertebrae at some point during the course of their illness. The highest percentages of cancers that lead to such spinal lesions are carcinomas of the breast, lung, prostate, colon, thyroid and kidney. This likely occurs through hematogenous spread. Pain is a common presenting symptom, but it may be found while still asymptomatic if routine screenings are done with bone scans, MRI or CT in patients with a known primary tumor elsewhere in the body. The pain is usually worse with weight bearing activities and better when lying down, but pain that persists through the night and prevents sleep needs to be evaluated for possible neoplasm. Some metastatic disease of the spine may present with neurological symptoms such as sensory or motor deficits following a spinal nerve root distribution or more generalized neurological deficits due to spinal cord compression or cauda equina syndrome.

What types of connective tissue are injured in a sprain?

A Bones and muscles
B Fascia and joint capsules
C Ligaments and joint capsules
D Muscles and tendons
E Tendons and bones

Ligaments and joint capsules
A sprain involves injury to those tissues that give support to joints - ligaments and joint capsules. Injury to muscles, tendons, and fascia would all be classified as a strain. Injuries to bone would be classified as a fracture.

What types of connective tissue are injured in a strain?

A Bones and muscles
B Fascia and joint capsules
C Ligaments and joint capsules
D Muscles and tendons
E Tendons and bones

Muscles and tendons
A strain involves injury to the muscles and tendons that are responsible for active movement of various body parts. Fascia is a part of the muscle-tendon unit, so injury to fascia would be considered a strain as well. Injury to ligaments and joint capsules would be considered a sprain and damage to bone would be classified as a fracture.

A 45-year-old nonsmoking female is found to have a 2-cm mass in the periphery of the left lung on a routine chest radiograph. She has no significant medical history, known exposures, or contributory family history. A subsequent biopsy confirms malignancy. Which of the following is the most likely underlying cell type?

A Squamous cell
B Large cell
C Adenocarcinoma
D Small cell

Adenocarcinoma
While adenocarcinoma, like other lung cancer types, is more likely to appear in smokers; it is the most common lung cancer seen in nonsmokers, particularly women and younger patients. It generally originates in the periphery of the lung. All other answers are associated with significant smoking history and, with the exception of large cell carcinoma, originate centrally.

A 64-year-old man has been experiencing signs and symptoms compatible with diverticular disease for the past 3 weeks. He now presents to the emergency department malnourished with severe left-sided lower abdominal pain. After appropriate workup and hydration, he is taken to the operating room where a perforated sigmoid colon is discovered with gross contamination. What is the most appropriate surgical intervention at this time?

A Left colectomy with primary anastomosis
B Hartmann procedure
C Proctocolectomy
D Abdominoperineal resection
E Low anterior resection

Hartmann procedure
This vignette is consistent with an emergent resection in an unprepared patient. The most appropriate therapy for an acute perforation is a Hartmann procedure, which includes resection of the affected portion of the bowel, a temporary diverting colostomy, and oversewing of the distal rectal stump; the second stage of the procedure will involve taking down the colostomy with anastomosis to the rectal stump. A colectomy with a primary anastomosis should not be done when the bowel is unprepared due to the significant risk of infection and leakage of the bowel at the site of the anastomosis. Abdominoperineal resection is used in the treatment of malignant disease of the lower rectum. In this procedure, a permanent colostomy is created and the entire rectum, anal canal, and anus are removed. In the management of benign disease of the lower rectum, a proctocolectomy is appropriate to preserve anal function.

A 60-year-old man presented with a mass in the left lobe of the thyroid. Fine needle aspiration was consistent with papillary carcinoma. There was no evidence of locally invasive or metastatic disease. Which of the following treatments is recommended for this patient?

A Chemotherapy
B External beam radiation
C Preoperative radioiodine ablation
D Total thyroidectomy

Total thyroidectomy
Papillary carcinoma is the most common type of thyroid malignancy. Treatment includes a thyroid lobectomy and isthmusectomy or total thyroidectomy. The decision regarding the extent of the surgery is based on the extent of the disease, the tumor size, and histiologic grade. A poor prognosis is seen in males, patients older than 50 years of age, primary tumors greater than 4 cm in size, tumors that are less well differentiated, or evidence of locally invasive or metastatic disease. Accordingly, the recommended treatment for this patient is a total thyroidectomy. Radioiodine ablation is recommended postoperatively.

A 39-year-old man presents to the emergency department with massive hematemesis. His physical examination reveals slight jaundice, palmar erythema, spider angiomas, and marked ascites. Vitals at the time of presentation are as follows: BP: 85/44 mm Hg, P: 122/min, R: 16/min, oxygen saturation: 96%, and T: 99.8°F. Which of the following is the most likely cause of the massive hematemesis?

A Peptic ulcer disease
B Mallory-Weiss tear
C Gastric carcinoma
D Arteriovenous malformation
E Esophageal varices

esophageal varices
The most common cause of massive upper gastrointestinal bleeding in patients with cirrhosis is esophageal varices. Although 20% of patients with portal hypertension will have bleeding from other causes (peptic ulcer disease, Mallory-Weiss tears, or gastritis), endoscopic evaluation in patients with portal hypertension is necessary for diagnosis and initial therapy.

A 48-year-old patient is monitored post-operatively for appropriate heparin dosing, and his aPTT is twice the upper limit of the therapeutic range. Which of the following can be administered to reverse the effect of his heparin overdose?

A Enoxaparin
B Fresh frozen plasma
C Protamine sulfate
D Recombinant factor VII
E Vitamin K

Protamine sulfate
Protamine sulfate is the reversal agent for heparin, but administration carries a significant risk of thrombosis and allergic reaction, and should be used cautiously when the benefits outweigh the risks.
Enoxaparin (A) should not be administered because it would be additive therapy to heparin. Fresh frozen plasma (B), recombinant factor VII (D), and vitamin K (E) are used in treating warfarin overdose.

When a bone is fractured, there are the 3 stages of healing. What is the proper order in which the healing occurs?

A Inflammatory, Remodeling, Reparative
B Inflammatory, Reparative, Remodeling
C Remodeling, Inflammatory, Reparative
D Remodeling, Reparative, Inflammatory
E Reparative, Inflammatory, Remodeling

Inflammatory, Reparative, Remodeling
Inflammatory changes happen after fractures and this is followed by a reparative phase and ultimately, a remodeling phase. The healing from acute injuries generally starts with inflammation,. Once the integrity of the bone has been restored on the macro level, bone remodeling continues until full healing has occurred.

A 6-year-old girl presents to the emergency department with abdominal distension of 1-day duration. She has not had a bowel movement or passed flatus in 72 hours. Examination reveals markedly diminished bowel sounds with tympany to percussion. She has also passed bloody mucus from her rectum. There is no evidence of hernia, and surgical history is negative. Which of the following is the most likely diagnosis?

A Regional enteritis
B Pyloric stenosis
C Meckel diverticulum
D Acute appendicitis

Meckel diverticulum
Meckel diverticulum is prevalent in 2% of the population, has a 2:1 male:female predominance, and is usually located 2 ft from the ileocecal valve. The most common clinical presentations are bleeding, intestinal obstruction, and inflammation. Bright red or maroon bleeding is the most frequent complication in children younger than 2 years of age. Obstruction may develop secondary to a volvulus that occurs at the site of the diverticulum or from an intussusception with the diverticulum acting as the lead point. An air contrast barium enema may be able to reduce intussusceptions in children.

A 29-year-old female has a long history of supraventricular tachycardia, for which she has been treated with long-term flecanide, as well as prior therapy with verapamil. She continues to have repeated episodes, sometimes two to three times a week, along with shortness of breath and at times hypotension that has been recorded. What is the next best therapy for this patient?

A Synchronized cardioversion
B Cardiac catheterization
C Ablation therapy
D Pacemaker insertion
E Long-term telemetry monitoring

ablation therapy
After exhaustion of non-invasive therapies, ablation therapy can be used to try to negate the aberrant pathway for SVT. Pacemakers will not allow for an override of the pathway, and cardioversion is only a temporary solution to an acute event. Implantable telemetry monitoring is only diagnostic and not therapeutic to treat.

What type of fracture is not related to an acute bony trauma?

A Greenstick
B Stress
C Oblique
D Comminuted
E Spiral

stress
A stress or fatigue fracture is caused by small, repetitive forces that usually involve the metatarsal shafts, the distal tibia, and the femoral neck (though many other bones may be affected). These fractures may not be seen on initial radiographs. A greenstick fracture is an incomplete traumatic fracture with angular deformity seen in children. An oblique fracture is a traumatic fracture with an angulated fracture line. A comminuted fracture is a traumatic fracture in which there are more than two fracture segments. A spiral fracture is a traumatic fracture that has a multiplanar and complex fracture line usually caused by an excessive rotational force on a bone.

A 22-year-old man is brought to the emergency department by paramedics after having sustained a single stab wound along the left sternal border at the fourth intercostal space. Upon arrival to the emergency department, he was hypotensive and tachycardic. The neck veins were distended and heart sounds were muffled. Which of the following interventions is the most appropriate first-line management of this patient?

A Left tube thoracostomy
B Pericardiocentesis
C Fluid resuscitation
D Immediate intubation

pericardiocentesis
Cardiac tamponade is classically described by the triad of jugular venous distension (JVD), arterial hypotension, and muffled heart sounds. In the emergency department, suspicion of this clinically entity is usually confirmed by ultrasonography and is acutely treated by pericardiocentesis, which will be diagnostic, therapeutic, and buy time until a definitive procedure can be done. A left tube thoracostomy may be indicated in this patient but would not relieve symptoms. Fluid resuscitation though applied to all trauma patients would help stabilize the patient until more therapeutic interventions could be completed. Immediate intubation, even if indicated, would require a prophylactic tube thoracostomy to prevent the development of tension pneumothorax in the event of an unrecognized lung injury. Emergency thoracotomy will relieve the signs and symptoms associated with cardiac tamponade and allow for repair of any underlying cardiac injuries.

A 70-year-old man with a history of hypertension, DM Type 2, and hyperlipidemia is seen for preoperative evaluation prior to left total knee replacement. On auscultation, a very soft high-frequency decrescendo early diastolic murmur is heard at the upper left sternal border. Utilizing isometric hand grip exercises, the murmur increases in intensity and can be heard radiating to the left sternal border and apex. Given the patient's physical exam findings, which of the following is the most appropriate next step in the management of this patient?

A Chest x-ray
B Transesophageal echocardiogram
C Holter monitor
D Treadmill exercise stress test
E Transthoracic echocardiogram

transthoracic echocardiograph
Choice E, transthoracic echocardiogram, is a simple, sensitive and non-invasive diagnostic tool which can evaluate for the presence of valvulopathy in this patient. Choice A might be able to give evidence of cardiomegaly, but would not be sensitive enough to detect valvulopathy. Choice B, transesophageal echocardiogram, would give information regarding valvulopathy, but is a more invasive test; therefore, choice E is more appropriate. Choice C is a useful diagnostic tool for evaluation of patients complaining of palpitations, but incorrect for this patient who has no symptoms. Choice D, although a useful diagnostic tool for the evaluation of exercise tolerance and in patients complaining of chest pain, does not allow direct visualization of the heart valves to evaluate for valvulopathy. In addition, a patient scheduled for left total knee replacement is unlikely to perform very well on a treadmill, thus the more appropriate test for preoperative evaluation, if necessary after transthoracic echocardiogram, would be a nuclear stress test.

A 56 year-old woman develops a fever of 102.1 F on her first post-operative surgery day following cholecystectomy. Which of the following is the most likely diagnosis?

A Atelectasis
B Catheter-related phlebitis
C Drug reaction
D Urinary tract infection
E Wound infection

atelectasis
Post-operative fever that occurs in the first 24 hours is most likely caused by atelectasis (A). Catheter-related phlebitis (B), drug reactions (C) and urinary tract infection (D) typically present between post-operative days 2 and 4, while wound infection (E) typically presents on or after post-operative day 5.

What is the most common site of an acute arterial occlusion due to embolic disease?

A Iliac artery
B Aortic bifurcation
C Mesenteric arteries
D Femoral artery
E Popliteal artery

femoral artery
The most common site for an acute embolic occlusion is the femoral artery. Other common sites include the axillary, popliteal, and iliac arteries as well as the aortic bifurcation and mesenteric vessels. The majority (80%) of arterial embolic originate in the heart in patients with atrial fibrillation or from mural thrombi in the left ventricle from an akinetic or dyskinetic portion of the myocardium following a myocardial infarction.

A 22-year-old male hurts his right knee while playing football on artificial turf. He states that he planted his foot and went to turn, but his leg didn't turn with his body. He instantly felt a popping sensation in the knee. A few hours later he develops an effusion. Based on the history of the injury, which knee structure was likely injured?

A Anterior cruciate ligament (ACL)
B Lateral collateral ligament (LCL)
C Medial collateral ligament (MCL)
D Posterior cruciate ligament (PCL)
E Quadriceps tendon

ACL
A Non-impact rotational or hyperextension forces are the most common mechanisms for sustaining a tear of the ACL. One third of patients report hearing an audible popping sound as their ACL tear occurred. Because the ACL is a vascular structure, when it tears a rapid bloody effusion (hemarthrosis) usually develops which effects mobility of the joint. Lateral collateral ligaments are the least likely to be injured as the type of force necessary to cause injury would be a varus stress which is unlikely to occur in typical circumstances. Medial collateral ligament injuries are fairly common and produced by a valgus force that stresses the ligament. This can occur in many sporting events including those in which another competitor might fall on or dive into the lateral aspect of the knee. Trauma to a knee can result in tears of both the ACL and MCL in certain situations. A tear of the quadriceps tendon usually occurs when a person falls on a knee that is partially flexed. As the quadriceps muscle contract to prevent excessive flexion, the force and momentum of the fall may overwhelm the knee extension mechanism and cause the rupture. No such mechanism occurred in our scenario. Posterior cruciate injuries occur when the tibia is driven posterior in relation to the femur as may happen when a car dashboard is driven into the tibias during a major front impact collision. A powerful hyperextension force can result in both ACL and PCL tears (usually in that order). PCL tears are much more uncommon than ACL tears and don't generally occur with basic rotational forces as described in our patient scenario.

A 58-year-old man presents with to the emergency department with the acute onset of the "worst headache of his life," which was associated with a brief loss of consciousness. The patient is complaining of increased pain with movement of his neck as well as photophobia. What is the recommended first-line study to evaluate this patient?

A Lumbar puncture
B Noncontrast CT scan of the head
C Magnetic resonance imaging (MRI) of the brain
D Four-vessel angiogram

noncontrast CT scan of the head
The initial study to diagnose a subarachnoid hemorrhage is a noncontrast CT scan of the head. If the CT scan is nondiagnostic for a SAH and the clinical suspicion is high, then proceed with a lumbar puncture for the presence of red blood cells in the cerebrospinal fluid; xanthochromia can be seen with an old SAH.

A 28-year-old woman presents with nervousness and palpitations associated with heat intolerance. On examination, there is no evidence of thyromegaly, but there is a palpable nodule that is "hot" on a thyroid scan. The TSH was low and T3 and T4 were both elevated. Which of the following is the recommended treatment for this patient?

A Propylthiouracil (PTU)
B Thyroid lobectomy
C Total thyroidectomy
D Radioiodine ablation

thyroid lobectomy
In Graves' disease, the thyroid is diffusely enlarged in contrast to a toxic adenoma in which the thyroid is normal sized but with a palpable nodule. Surgery is the treatment of choice for a toxic adenoma. Surgical treatment of a toxic adenoma is a thyroid lobectomy and isthmusectomy. A subtotal or total thyroidectomy is indicated for toxic multinodular goiters or Plummer disease. Thionamides and radioiodine ablation are not effective therapies for toxic adenomas. (Coe, 2006, pp. 404-406) Coe NPW. Surgical endocrinology: thyroid gland.

A 64-year-old man has been experiencing intermittent left lower abdominal pain associated with alternating diarrhea and constipation. The pain has been increasing over the past 24 hours and is now associated with a fever. The abdomen is tender with evidence of peritoneal signs. Which of the following diagnostic studies is most appropriate to evaluate this patient?

A Barium enema
B Computed tomography (CT)
C Sigmoidoscopy
D Colonoscopy

CT
For a patient with diverticular disease, the preferred study to evaluate complications, such as a perforation or abscesses, is a CT scan. A barium enema or endoscopic procedure is contraindicated due to increased risk of perforation during an acute exacerbation.

What is the most common joint dislocation in children?

A Ankle
B Elbow
C Finger
D Knee
E Shoulder

elbow
In children the elbow is the most commonly dislocated joint and it is the third most common joint dislocation for adults. The shoulder and finger are dislocated more frequently than the elbow in adults. Knee dislocations at the femur-tibia joint are rare (patellofemoral dislocations are more common) and ankle dislocations are also relatively rare.

Following a total thyroidectomy for papillary carcinoma, a 72-year-old man develops a heart rate of 140 and a temperature of 104.8F. He vomits almost continuously and has severe diarrhea. He is disoriented and mildly combative. Electrocardiography demonstrates sinus tachycardia. What is the most likely diagnosis?

A pulmonary embolism
B pneumonia
C sepsis
D thyroid storm
E wound infection

thyroid storm
Although rare, thyroid storm or crisis can occur following thyroid surgery, administration of radioactive iodine, or a stressful illness. It is characterized by extreme tachycardia, vomiting, diarrhea, dehydration, delirium, and high fever. Pulmonary embolism (A) may include tachycardia, along with chest pain and shortness of breath, but is unlikely to include GI symptoms. Pneumonia (B) and sepsis (C) are certainly possible, although the extreme nature of her findings more strongly suggest thyrotoxicosis. Wound infection (E) would more likely to present with localized tenderness and a less marked fever.

A 32-year-old lactating female presents to the surgical clinic with a fluctuant mass of her left breast. The area directly above the lesion is erythematous and tender to touch. You make the diagnosis of localized breast abscess. Which of the following pathogens is the most likely cause of the patient's symptoms?

A Staphylococcus aureus
B Viridens streptococcus
C Pseudomonas aeruginosa
D Escherichia coli

staph aureus
A breast abscess is commonly seen in conjunction with lactation. The most common bacterial pathogen is Staphylococcus aureus. While antibiotics are the treatment of choice, the patient should be instructed to continue breast feeding in order to promote drainage of the breast. If the abscess does not respond to antibiotics, then an aspiration should be considered.

Out of all cervical vertebrae, which two are responsible for the greatest amount of rotation?

A C1 & C2
B C2 & C3
C C3 & C4
D C4 & C5
E C5 & C6

C1 & C2
Approximately 50% of cervical rotation takes place between the C1 (atlas) and C2 (axis) vertebrae. These first two cervical vertebrae have a different shape from the other cervical vertebrae that allow for this greater range of motion. The remaining 50 % of cervical rotation is split fairly evenly between the remaining vertebrae. Approximately 50 % of flexion and extension occurs between the occiput at the base of the skull and C1 with the remaining 50% distributed fairly evenly between the remaining vertebrae with a slightly higher percentage occurring at the C5 & C6 level.

A patient presented with intermittent rectal bleeding associated with decreased caliber in the size of his stool. On examination, there were no palpable abdominal masses, but the fecal occult testing was positive. A barium enema was obtained with the results pictured below. What is the most likely diagnosis for this patient?

A Diverticular disease
B Crohn disease
C Colorectal carcinoma
D Intussusception

Colorectal cancer
Barium enema finding of carcinoma of the sigmoid colon causing high-grade obstruction shows the classic "apple core" lesion. Crohn's disease is typically associated with the string sign, which is an area of stricture or stenosis that shows up as a narrow line of contrast, giving the appearance of a string associated with the stricture. Diverticular disease is associated with outpouchings from the colon that will be filled with barium.

A 54-year-old man with a history of chronic alcohol abuse presents to the emergency department with complaints of a subjective fever and severe epigastric pain radiating to the back. The pain has been present for the past 8 hours and is associated with nausea and vomiting, which has not relieved the pain. Laboratory data reveal a WBC of 14,000/mm 3 and a serum amylase of 500 U/L (reference range 0-286 U/L). Plain films of the abdomen were unremarkable. Which of the following is the most likely diagnosis?

A Perforated duodenal ulcer
B Acute cholecystitis
C Acute pancreatitis
D Mesenteric ischemia
E Choledocholithiasis

acute pancreatitis
Acute pancreatitis typically presents with severe, steady midepigastric abdominal pain that radiates through to the back; pain is associated with fever, nausea, and vomiting. The most common causes of acute pancreatitis are gallstones and alcohol. Laboratory studies will show elevated WBC and serum amylase levels. Amylase elevations are nonspecific and can be elevated with perforated ulcers and mesenteric ischemia. A perforated ulcer will show evidence of free air on plain film; mesenteric ischemia will not present with fever or an elevated WBC unless there is the presence of infarcted bowel at which point the patient would appear septic. Acute cholecystitis may be associated with elevations in amylase but they are typically only a modest increase

The rotator cuff is comprised of which four muscles?

A Scalenes, infraspinatus, teres minor, subscapularis
B Supraspinatus, infraspinatus, teres major, subscapularis
C Supraspinatus, infraspinatus, teres minor, soleus
D Supraspinatus, infraspinatus, teres minor, subscapularis
E Supraspinatus, intercostals, teres minor, subscapularis

Supraspinatus, infraspinatus, teres minor, subscapularis
Only option D lists the correct muscles of the rotator cuff located in the shoulder area that contribute to arm elevation, internal rotation and external rotation. Of the teres muscle group, it is the teres minor, not teres major that is part of the rotator cuff. The scalene muscles are in the neck and cause rib elevation and neck movement. The soleus muscles cause plantar flexion of the feet and are located in the calf area of the posterior lower leg

A fracture involving the medial epicondyle will most likely cause damage to which nerve?

A Axillary
B Median
C Peroneal
D Radial
E Ulnar

ulnar
Because the ulnar nerve passes through the cubital tunnel, which is a groove on the posterior aspect of the medial epicondyle, any fractures involving the medial epicondyle can also cause damage to the ulnar nerve. The median nerve is most susceptible to injury at the carpal tunnel. Branches of the radial nerve can become entrapped on the lateral side of the elbow and the associated symptoms are often confused with lateral epicondylitis. Radial nerve injuries are more commonly associated with humeral shaft fractures. The axillary nerve is significantly proximal to the medial epicondyle and the peroneal nerve is in the leg

A 46-year-old male is evaluated for a fall from approximately 15 feet, landing on his legs. There is intense pain and swelling to the lower extremity, with decreased sensation. What bone is fractured and is most commonly associated with acute compartment syndrome?

A Femur
B Patella
C Navicular
D Talus
E Tibia

tibia
The tibia is the most common bone fracture that leads to acute compartment syndrome. It can also occur after other long bone fractures of the arms and legs. While it does occur in the hands and feet, it is unlikely that the fracture of a small carpal bone (scaphoid) or tarsal bone (talus) would result in compartment syndrome. Damage to the hyoid bone in the neck and the patella in the knee are not associated with compartment syndrome.

Which of the following is a type of primary malignant tumor of the bone?

A Enchondroma
B Leiomyoma
C Lipoma
D Melanoma
E Osteosarcoma

osteosarcoma
Osteosarcoma is a type of primary bone cancer. Fortunately this is a rare cancer, accounting for only 1% of the cancers diagnosed each year in the USA for patients of all ages. Osteosarcomas are more common in the pediatric population, accounting for 5% of all childhood cancers and 56% of all cancers of the bone in patients less than 20 years old. Osteosarcomas have a bimodal occurrence rate with the highest prevalence between ages 11-13 and over the age of 65. Enchondromas are common benign cartilaginous tumors that develop in the medulla (marrow cavity) of bone. A leiomyoma is another name for a benign uterine fibroid. A lipoma is a benign, soft, freely movable, generally nontender mass in the soft tissue sometimes referred to as a fatty tumor. These are generally inconsequential, but may be a marker for spina bifida if found in the lumbar region. Melanoma can be found in bone, but it would be considered a metastatic lesion and not a primary malignant bone tumor

Which of the following would raise your suspicions the most and likely warrant consideration of testing for an inherited thrombophilia?

A a deep femoral vein deep vein thrombosis (DVT) after a flight from Mumbai, India
B an iliac vein DVT after a round trip bus trip to Atlantic City and playing slots all day
C any DVT after a total knee replacement
D an upper extremity DVT after tripping falling down a flight of stairs
E any DVT in a woman who recently started oral contraceptives and smokes

an upper extremity DVT after tripping falling down a flight of stairs
DVTs most commonly arise from the deep femoral veins and iliac arteries, most commonly in patients who smoke and take oral contraceptives, after immobilizing surgeries, and/or after immobilization due to long periods of time seated, including but not limited to airplane flights, bus rides, etc. Upper extremity DVTs are rare, even after trauma, and warrant a hypercoaguability work-up to rule out inherited disease.

A 52-year-old male has a 200-pound file cabinet fall on his right leg. He comes to the emergency department complaining of pain and swelling to the right leg. He is also complaining of parasthesias to the leg also. Based on these findings on history, what part of the leg would be the most likely site of compartment syndrome?

A Anterior compartment of the leg
B Deep posterior compartment of the leg
C Dorsal compartment of the forearm
D Lateral compartment of the leg
E Superficial posterior compartment of the leg

Anterior compartment of the leg
Anterior compartment syndrome is most commonly found in the anterior compartment of the leg, with the volar compartment of the forearm also a common location. The mechanism is generally an acute crushing trauma to the affected area that causes an increase in pressure within the compartment that inhibits venous outflow and a decrease in arterial blood flow. This adversely affects tissue perfusion and ischemia of the involved tissues can occur. The symptoms can also occur with chronic exertion or when there is a dramatic increase in the amount of exercise being performed. Anterior compartment syndrome of the leg is likely most common due to its vulnerable location and susceptibility to injury in athletics and motor vehicle accidents. The rate of occurrence of acute anterior compartment syndrome is also likely due to the fact that the anterior compartment lies adjacent to the tibia and the tibia is the most frequently fractured long bone. Compartment syndrome could occur in any compartment of the upper or lower extremities if the necessary circumstances were in place (either acute trauma to the area or excessive use of the muscles that are associated with each of the compartments).

A 59-year-old woman presents to the emergency department with an acute upper gastrointestinal hemorrhage. Her medical history is pertinent for peptic ulcer disease for the past 5 years and hypertension. A nasogastric tube is inserted and bright red blood is seen. Her vital signs are BP: 110/70 mm Hg, P: 94/min, R: 14/min, oxygen saturation: 97%, T: 99°F. Which of the following diagnostic studies would be the most appropriate next step to determine the site of bleeding?

A Abdominal and pelvic computed tomography
B Abdominal ultrasound
C Upper gastrointestinal series with barium
D Bleeding scan
E Upper endoscopy

upper endoscopy
Patients who present with upper gastrointestinal bleeding and shock requiring multiple transfusions in 24 hours are at high risk for mortality from gastrointestinal bleeding. The upper gastrointestinal bleeding in this patient warrants further investigation with upper gastrointestinal endoscopy to both determine the site of bleeding and provide potential therapy by endoscopic electrocautery or injection.

A 46-year-old African American male is seen in the emergency department with upper right quadrant pain that radiates to the right infrascapular area. The pain is colicky and was precipitated by a meal of fried fish and French fries. Which of the following diagnostic studies is the initial study of choice for this patient?

A Plain abdominal x-ray
B Ultrasonography
C Radionuclide scan (HIDA scan)
D Computed tomography (CT)

ultrasonography
Ultrasonography is the first-line study in the evaluation of patients presenting with signs and symptoms of biliary disease. The sensitivity and specificity is 95%. It can detect stones, dilation of biliary ducts, thickening of the gallbladder, and pericolic collections of fluid and can also provide information pertaining to associated liver or pancreatic pathology.

A 58-year-old man presents with the acute onset of abdominal pain associated with fever and shaking chills. The patient is hypotensive and febrile with a temperature of 102.2°F. Although he is confused and disoriented, he complains of right upper quadrant pain during palpation of the abdomen. His sclerae are icteric and the skin is jaundiced. Which of the following is the most likely diagnosis?

A Acute cholecystitis
B Choledocholithiasis
C Acute pancreatitis
D Ascending cholangitis

ascending cholangitis
The presenting symptoms associated with ascending cholangitis include fever, chills, right upper quadrant pain, and jaundice (Charcot's triad); the symptoms are secondary to an infected obstruction of the common bile duct. With spread of the infection, the patient may also develop hypotension and mental status changes; these additional symptoms in conjunction with Charcot's triad are known as Reynolds' pentad. Additional symptoms of common bile duct obstruction include light-colored stools and dark, tea-colored urine.

A 62-year-old man presents to the office concerned about an abdominal aortic aneurysm (AAA). He has had no symptoms but states that his father died from an aortic dissection at the age of 50 and his brother was diagnosed last week with an AAA. What is the most appropriate screening tool in this situation?

A Abdominal radiograph
B Computed tomographic angiography (CTA)
C Palpation
D Ultrasound
E Aortography

ultrasound
Ultrasonography is cost-effective and is the most commonly utilized screening modality for AAAs. It can be utilized for initial detection of a nonruptured AAA and for monitoring of progression. Anteroposterior and lateral abdominal radiographs may reveal calcification of an AAA as an incidental finding but are nonsensitive/nonspecific. CTA scans are used in monitoring for progression of AAA size and preoperatively to assess aortic anatomy. Abdominal palpation on physical examination may be reliable in thin patients but cannot accurately provide information about the presence or absence of aneurysms in all patients. Aortography is an expensive and invasive procedure traditionally used in preoperatively planning of AAA repair; however, it has largely been supplanted by multiplanar CTA scans.

Which of the following is a major contraindication for surgical resection of a lung carcinoma?

A Chest wall invasion
B Non-malignant pleural effusion
C Superior vena cava syndrome
D Unilateral endobronchial tumor
E Vagus nerve involvement

superior vena cava
Surgical resection of lung carcinoma is contraindicated in cases of superior vena cava syndrome, extrathoracic metastases, heart, pericardial or great vessel involvement, recurrent laryngeal or phrenic nerve involvement, esophageal or carina involvement, malignant effusion, or contralateral mediastinal lymph nodes. Other contraindications are patient and staging dependent.

A patient presents with abdominal distension associated with nausea and vomiting. Which of the following findings is consistent with a paralytic ileus?

A Crampy abdominal pain
B Hyperactive bowel sounds
C Obstipation and failure to pass flatus
D Gas in small intestine only on KUB (kidney, ureter, bladder)

Obstipation and failure to pass flatus
Obstipation and failure to pass flatus are actually symptoms of both paralytic ileus and a small bowel obstruction (SBO). However, patients with a paralytic ileus usually have minimal abdominal pain and hypoactive or absent bowel sounds due to hypomotility. Patients with an SBO will have crampy abdominal pain and increased bowel sounds with high-pitched sounds and rushes due to increased peristalsis. Plain films in paralytic ileus will show gas throughout the small and the large bowel on plain films as opposed to air confined to the small intestine only in SBO

A 75-year-old woman with a history of hypertension is seen for preoperative evaluation prior to bladder suspension. On auscultation, a crescendo-decrescendo systolic ejection murmur is heard at the upper right sternal border, radiating to the carotids bilaterally. Given the patient's physical exam findings, which of the following is the most likely diagnosis?

A Aortic stenosis
B Aortic regurgitation/insufficiency
C Mitral stenosis
D Tricuspid regurgitation/insufficiency

Aortic stenosis
Choice A is correct, as the murmur of aortic stenosis is usually described as a crescendo-decrescendo or systolic ejection murmur that is heard best at the right upper sternal border. In addition, the murmur of aortic stenosis is frequently transmitted to the carotid arteries. Choice B is incorrect, as the murmur of aortic regurgitation is usually described as a high-frequency decrescendo early diastolic murmur that is heard best at the left upper sternal border or at the right upper sternal border. Choice C is incorrect, as the murmur of mitral stenosis is described as a low-frequency rumbling diastolic murmur that is decrescendo in early diastole, but may become crescendo up to the first heart sound, with moderately severe mitral stenosis and sinus rhythm. Choice D is incorrect, as the murmur of tricuspid regurgitation is described as a holosystolic descrescendo murmur.

Tumor necrosis factor (TNF) inhibitors are most often considered for use in patients with rheumatoid arthritis (RA) that does not respond to initial therapy. Which of the following screenings should occur before a patient is placed on this class of medication?

A chest x-ray
B allergy testing
C liver function tests
D purified protein derivative (PPD) test
E serum BUN (blood urea nitrogen) and creatinine test

PPD test
Patients being treated for rheumatoid arthritis with tumor necrosis factor (TNF) inhibitors are at increased risk for developing an opportunistic infection, such as tuberculosis (TB). It is recommended that screening for the presence of latent TB occur before TNF inhibitors are started. There is no specific indication to order a chest x-ray, allergy testing, liver function tests, or serum BUN and creatinine prior to initiation of TNF inhibitors

The most commonly fractured long bone in both adults and children is which of the following?

A Femur
B Fibula
C Humerus
D Radius
E Tibia

Tibia
The tibia is the most commonly fractured long bone in the body for both adults and children. The fractures are often the result of sporting activities in the young and may occur from a simple fall in the elderly - especially those with osteoporosis. Motor vehicle accidents are another common cause of tibial fractures. Open or complex tibial fractures are sometimes associated with compartment syndromes, infection and neurovascular compromise. The femur is the strongest of the long bones and generally only sustains fractures when exposed to extreme stress, such as that experienced in a motor vehicle collision or industrial accident. Fibular fractures commonly occur with a direct below to the lateral lower leg or with extreme ankle rotational forces or excessive inversion. A high percentage of ankle fractures involve the fibula, especially in older women. Humerus fractures are relatively rare in adults, but are the second most common fractures to occur at birth - behind only the clavicle in frequency. The radius is the most commonly fractured bone in the upper extremity, but still less common in frequency than the tibia. Falls on an outstretched arm are a common mechanism for the injury.

A 12-year-old girl presents with complaints of intermittent pain and stiffness involving her hands. This pain has been progressively worsening over the past 3 years. She relates that for the past 2 months she has been feeling increasingly tired and has experienced swelling and stiffness of her hands, which appears worse in the morning and is relieved as the day progresses. The physical examination shows she has a low-grade fever. There are multiple symmetrical joint swelling of the proximal interphalangeal and metacarpophalangeal joints with associated warmth, tenderness, and effusion. Initial laboratory findings include a CBC that reveals mild anemia, an elevated erythrocyte sedimentation rate, a positive rheumatoid factor, and a negative antinuclear antibody (ANA) test. X-rays of the hands and wrists show soft tissue swelling and periarticular osteopenia. Which of the following is the most likely diagnosis?

A reactive arthritis
B infectious arthritis
C systemic juvenile rheumatoid arthritis
D polyarticular juvenile rheumatoid arthritis
E pauciarticular juvenile rheumatoid arthritis

The most likely diagnosis in this patient is polyarticular juvenile rheumatoid arthritis (JRA).
This form of JRA is seen in approximately 35% of patients with JRA. It is characterized by symmetrical involvement of five or more joints. Two subsets of the disease exist that are distinguished by the presence or absence of rheumatoid factor. A positive rheumatoid factor is most commonly seen in girls with later disease onset (at least 8 years old). An antinuclear antibody (ANA) test may be positive but is more likely to be positive with the pauciarticular form. In the early stage of the disease, the x-ray may be normal or show soft tissue swelling and periarticular osteopenia. In addition to the positive ANA of pauciarticular JRA patients, the arthritis must be present in four or fewer joints. Early onset disease is commonly seen in girls aged 1 to 5 years and has a positive ANA; up to 30% of patients will also have eye involvement. Late onset disease is more common in male patients, with involvement of the large joints. Systemic JRA, also known as "Still disease," is seen in about 10% to 15% of children with JRA. It is characterized by daily intermittent fever spikes and a transient, nonpruritic, pale pink, blanching macular, or maculopapular rash found on the trunk. A positive rheumatoid factor is rare in this form of JRA. Reactive arthritis is usually associated with a recent viral or bacterial infection. Infectious arthritis more commonly presents as monarticular and is usually acute in onset.

A 56-year-old male who works in construction climbing ladders has developed pain to the right foot for several days. You have seen and examined the patient a few days after the patient started complaining of pain to the foot. Your initial x-rays of the foot are negative for fracture. He continues to have pain, and decreased ability to bear weight. Based on this clinical scenario, how many days after the initial examination should another x-ray be ordered to look for a stress fracture?

A 1-2 days
B 5-7 days
C 10-12 days
D 14-30 days
E 180 days

14-30 days
Radiographic evidence of stress fractures is not immediately apparent after the initial onset of symptoms. Estimates of anywhere from 14 to 42 days before visible signs of a stress fracture can be detected on X-ray have been put forth with a commonly utilized range of 14 to 30 days. Any answer choice above that included numbers less than 14 days would simply be inaccurate due to the gradual appearance of the classic x-ray findings of a stress fracture. After 180 days from the time of stress fracture onset, the fracture should be nearly healed if managed properly. If a stress fracture is highly suspected in light of negative x-rays, imaging with a bone scan should help make the diagnosis. MRI scans may help confirm the diagnosis, but they are rarely utilized in the work-up of suspected stress fractures.

A 52-year-old man with hypertension associated with recent unexplained weight loss presents with fever, malaise, and gradual onset of pain and weakness of his leg muscles for the past month. Physical examination reveals a mottled reticular pattern overlying portions of both calves and an area of ulceration with surrounding induration on the left lateral malleolus. Initial laboratory results reveal mild normochromic anemia, leukocytosis, and elevation of C-reactive protein, BUN, and creatinine. Which of the following is the most appropriate diagnostic evaluation to confirm the suspected diagnosis?

A HLA-B27 typing
B rheumatoid factor
C MRI of sacroiliac joints
D antinuclear antibodies test
E tissue biopsy of area of induration

tissue biopsy of area of induration
This patient most likely has polyarteritis nodosa (PN). A major obstacle in making the diagnosis is the absence of a disease-specific serological test. The diagnosis requires confirmation with either a tissue biopsy or angiogram. HLA-B27 antigens are not associated with the suspected diagnosis. While classic PN will have low titers of rheumatoid factor and antinuclear antibodies, both are nonspecific findings and will not confirm the diagnosis. An MRI of the sacroiliac joints is indicated in evaluation of the early stages of suspected ankylosing spondylitis and plays no role in the evaluation of PN.

Radiculopathy due to nerve root compression occurs most commonly at which nerve root within the brachial plexus?

A C5
B C6
C C7
D C8
E T1

C7
The C7 nerve root is affected the most often (approximately 45-60%). This radiculopathy can result from foraminal encroachment of the spinal nerve, cervical disk herniation, tumor, and multiple sclerosis. C7 radiculopathy can present with weakness in the triceps, which cause elbow extension, and finger flexion and extension. C6 is another common site of radiculopathy. C6 radiculopathy can present with weakness in the biceps, brachioradialis, and wrist extensor muscles. Cervical radiculopathy at the C5, C8, and T1 are less common, but still possible. C5 radiculopathy can present with deltoid and biceps muscle weakness. C8 radiculopathy can present with finger flexor weakness and T1 radiculopathy with finger abduction weakness.

Upon testing a patient for function of the hip abductors, which muscle is considered the primary muscle responsible for most abduction?

A Biceps femoris
B Gluteus maximus
C Gluteus medius
D Gluteus minimus
E Vastus medialis

gluteus medius
The primary mover in the motion of hip abduction is the gluteus medius muscle. Gluteus minimus does play a supporting role in that motion. Biceps femoris is one of the three hamstring muscles and contributes to the motions of knee flexion (primary muscle) and hip extension (secondary muscle). Gluteus maximus is the primary mover for hip extension and vastus medialis is one of the four quadriceps muscles responsible for knee extension, but no hip movements.

How many types of primary osteoporosis are there?

A 1
B 2
C 3
D 4
E 5

2
Osteoporosis is a condition characterized by low bone mass, which increases the fragility of bones and leads to an increased risk of fracture. Osteoporosis is defined as being either primary or secondary and primary osteoporosis is further broken down into Type 1 and Type 2. Type 1 is related to decreased hormone levels - estrogen in women and testosterone in men, and is sometimes referred to as "postmenopausal osteoporosis." It is six times more common in women and results in loss of trabecular bone. Type 1 primary osteoporosis often presents with vertebral compression fractures or fractures of the distal radius after a fall. Type 2 primary osteoporosis is sometimes referred to as "senile osteoporosis" and generally occurs in patients over 70 years of age. It is twice as common in women as men and occurs due to a diminished capacity to make new bone. The most common types of fractures found in this type of osteoarthritis are hip and pelvic fractures. Secondary osteoporosis occurs at a somewhat higher rate in men versus women and is caused by some other medical condition that produces bone loss. Common causes include long-term steroid use, various endocrine abnormalities, and neoplastic diseases such as multiple myeloma

What age group is most at risk to develop osteoid osteoma?

A Adolescents
B Elderly
C Middle-aged
D Neonates
E Toddlers

adolescents
Osteoid osteoma is a benign bone forming tumor that usually develops during a patient's second decade of life. This type of tumor is much more common in boys than girls and typically affects the lower extremities (femur and tibia primarily) and spine more than other areas of the body. Patients typically present with gradually progressive bone pain that is worse at night and does not correlate with activity level. The tumor produces high levels of prostaglandins, so symptoms usually improve in 20-25 minutes if the patient takes a medication like ibuprofen, ASA or other NSAIDS that are prostaglandin inhibitors. A lack of improvement in symptoms with these medications should lead health care providers to consider a different diagnosis. The pain of this condition may cause those afflicted in a leg to limp and have swelling, muscle atrophy or contractures and exquisite point tenderness. The condition usually resolves on its own over time, but symptomatic patients may require surgical resection or radioablation of the tumor

A 32-year-old female professional golfer presents with a 3-week history of pain along her thumb and down her wrist. She denies any trauma and states that it is aggravated with any movement of her wrist and thumb. Her physical examination is unremarkable. You perform the following test:
This test requires her to cup her thumb in a closed fist and ulnar deviate, which reproduces her pain. Considering the suspected diagnosis based on the positive examination above, what are the affected anatomical structures?

A Extensor pollicis brevis and abductor pollicis longus
B Flexor pollicis longus and flexor digitorum profundus
C Extensor carpi radialis longus and brevis
D Ulnar collateral ligament

Extensor pollicis brevis and abductor pollicis longus
De Quervain tenosynovitis is a common condition that occurs in patients who have experienced excessive use of the thumb or wrist. This is a tenosynovitis of the extensor pollicis brevis and abductor pollicis tendons (A), where the tendons lie in the groove of the radial styloid. The diagnosis of De Quervain tenosynovitis is supported by a history of pain in this location along with a painful range of motion of the thumb and further confirmation may be provided by a positive Finkelstein test. The flexor pollicis longus and flexor digitorum profundus (B) are affected in carpal tunnel syndrome. Extensor carpi radialis longus and brevis (C) are extrinsic extensor muscles of the hand. The ulnar collateral ligament (D) is injured in Gamekeeper's thumb.

A 36-year-old auto mechanic presents to the emergency department after hurting his back on the job. While lifting an object, he experienced sudden pain in his lower back with radiation to the right buttock. He was initially treated for muscle strain with a nonsteroidal anti-inflammatory drug (NSAID) after x-rays of his lumbosacral spine demonstrated no pathology. He continued to complain of this low back pain now radiating posteriorly down his left leg to the mid-thigh. Physical examination is unremarkable. The most likely diagnosis is

A lumbosacral strain
B left S1 radiculopathy
C cauda equina syndrome
D L5-S1 disc herniation
E lateral femoral cutaneous neuropathy

lumbosacral strain
Low back pain is one of the more common presenting neurologic complaints to a primary care provider. Most acute pain syndromes are benign, self-limiting conditions, with pain arising from myofascial sources. Patients with back pain and normal neurologic examinations are unlikely to have any serious underlying pathology and further diagnostic testing is usually unrevealing.

A 23-year-old patient who has recently been on a ski trip presents with pain to the right hand after sustaining a fall. It is difficult to move, and there is pain on flexion of the digit. Based on this history what ligament would the patient most likely have injured?

A 1st MCP joint ulnar collateral ligament
B 2nd MCP joint ulnar collateral ligament
C 3rd MCP joint ulnar collateral ligament
D 4th MCP joint ulnar collateral ligament
E 5th MCP joint ulnar collateral ligament

1st MCP joint ulnar collateral ligament
The ulnar collateral ligament at the base of the thumb, or 1st MCP joint, is often injured in forced abduction, such as a fall while skiing or during other sporting activities. An injury to this ligament has traditionally been called Gamekeeper's Thumb, but the origin of this term referred to a more chronic injury sustained by English gamekeepers as a result of the way they killed rabbits using their hands. Any of the MCP joint ulnar collateral ligaments could be injured in a fall if the mechanism of injury creates significant forces on the ligaments, but the 1st MCP joint is far more commonly injured than the others mentioned above.

What is the first type of motion that is lost as a person develops progressively worsening osteoarthritis of the hip joint?

A Abduction
B Adduction
C External rotation
D Flexion
E Internal rotation

internal rotation
The earliest sign of the development of osteoarthritis of the hip is often the loss of internal rotation. As the condition worsens, muscular contractures may develop which hold the affected limb in a flexed and externally rotated position, which has great consequence on the individual's gait and functioning level. Typically a person experiencing this type of osteoarthritis will eventually develop an antalgic gait where the time spent bearing weight on the affected limb is brief due to pain. Also, the gluteus medius (which is a hip abductor and helps stabilize the pelvis) may become weakened as the condition worsens, resulting in an abductor lurch as the trunk of the body sways out over the affected limb when attempting to walk

Patients in which of the following age groups are least likely to experience a dislocation or sprain when a significant stress is placed on their joints?

A 5-10 years old
B 15-20 years old
C 35-40 years old
D 55-60 years old
E 75-80 years old

5-10 yo
Dislocations and ligamentous injuries are uncommon in prepubertal children as the ligaments and joints are quite strong as compared to the adjoining growth plates. Excessive force applied to a child's joint is more likely to cause a fracture through the growth plate than a dislocation or sprain

A 65-year-old man presents with complaints of acute onset of pain and swelling of the right great toe. He denies recent alcohol ingestion or trauma to the area. On physical examination, the patient is afebrile, and the first metatarsophalangeal joint is erythematous, swollen, and warm to the touch. Laboratory evaluation reveals a WBC (white blood cells) count of 12,000/μL and a normal differential. Serum uric acid level is found to be 5 mg/dL. Synovial fluid analysis reveals the presence of rhomboid-shaped crystals. Which of the following is the most likely diagnosis?

A acute gout
B pseudogout
C psoriatic arthritis
D infectious arthritis
E rheumatoid arthritis

pseudogout
Pseudogout presents similarly to acute gout and is best diagnosed by the finding of the rhomboid-shaped crystals of calcium pyrophosphate in joint aspirates. Joints commonly involved in pseudogout are the knees and wrists and other joints such as the metacarpophalangeals, hips, shoulders, ankles, and elbows. The diagnosis of pseudogout is further supported by the finding of a normal serum uric acid level. Acute gout would more likely be associated with an elevated serum uric acid level. Psoriatic arthritis commonly presents with asymmetrical oligoarticular involvement of two to four joints, and in a higher percentage of patients, there is known presence of the dermatological expression of psoriasis. Infectious arthritis is ruled out with the findings of an afebrile patient and WBC count of 12,000/μL. In acute infectious arthritis, the WBCs would be expected to be elevated in the range of 50,000 to 200,000/μL. Rheumatoid arthritis usually presents with symmetrical polyarticular involvement of three or more joints.

A 35-year-old male presents with pain and decreased range of motion after sustaining a fall in which the patient tried to grab onto a bar which pulled his entire arm in the process. Given this clinical scenario, at what cervical motor neuron level would the biceps reflex be testing?

A C4
B C5
C C6
D C7
E C8

C5
C5 is the primary motor neuron being tested in a biceps reflex. C6 contributes to the brachioradialis reflex primarily, but does have a small role in the biceps reflex. The C7 motor neuron is primarily involved in the triceps reflex. C4 and C8 do not contribute to any primary reflexes

A disk herniation that is putting pressure on the L5 nerve root may present with weakness of what muscle(s)?

A Anterior tibialis
B Extensor hallucis longus
C Gastrocnemius-soleus
D Iliopsoas
E Peroneus longus and brevis

Extensor Hallucis longus
The extensor hallucis longus muscle's motor function is associated the L5 motor neuron, which also supplies the gluteus medius and extensor digitorum longus and brevis muscles. The anterior tibialis muscle is supplied by the L4 motor neuron. Nerves emanating from T12, L1, L2 and L3 supply the iliopsoas. Gastrocnemius, soleus and peroneus longus and brevis are all supplied by nerves coming from the S1 area. The plantar flexing gastrocnemius and soleus muscles also are supplied by S2.

A 78-year-old Caucasian female has a 3-year history of stiffness and achiness of bilateral shoulders and hips. She has been tested for rheumatoid arthritis in the past and has been found negative. Multiple radiographs of her hips and shoulders are unremarkable. She admits that she was placed on prednisone for an allergic reaction and noted a temporary resolution of her symptoms. For the past two weeks she complains of increasing symptoms now involving her neck and pain in her jaw with chewing. Today she noticed that her scalp is sore when she brushed her hair on the right side. What is the most feared complication of this condition that may be prevented with prompt diagnosis and treatment?

A blindness
B costovertebral angle (CVA)
C aneurysm
D arm claudication
E polymyalgia rheumatica (PMR)

blindness
The correct answer is (A). This patient has long standing symptoms of PMR with current symptoms suggestive of giant cell (temporal) arteritis. Visual loss is the most feared complication of temporal arteritis, but it can be prevented by prompt initiation of high-dose prednisone. PMR often occurs with or prior to development of temporal arteritis and is not considered a complication. Large vessel involvement--which may result in choices (B), (C), and (D)--is less common than temporal artery involvement in GCA. The patient does not have symptoms of large vessel involvement.

A 48-year-old woman presents with a chief complaint of gradually progressing difficulty in climbing stairs over the past 3 months. The physical examination shows there is notable proximal muscle weakness of the upper and lower extremities. The remainder of the examination is unremarkable. The laboratory evaluation shows an elevated serum creatinine phosphokinase level, and a muscle biopsy reveals lymphoid inflammatory infiltrates. Which of the following is the appropriate initial treatment of choice in this patient?

A prednisone
B azathioprine
C methotrexate
D immunoglobulin
E hydrochloroquine

prednisone
The most likely diagnosis in this patient is polymyositis. This is supported by the finding of a gradual progressive proximal muscle weakness and elevation of creatinine phosphokinase level. The finding of lymphoid inflammatory infiltrates on muscle biopsy confirms the diagnosis. Initial treatment of choice in this condition is the use of a corticosteroid (prednisone). Patients who do not respond to prednisone may then benefit from the use of methotrexate or azathioprine. Both intravenous immune globulin and hydroxychloroquine are effective for the treatment of patients with dermatomyositis that is resistant to prednisone therapy.

A 65-year-old woman presents with severe mid-back pain of 2 weeks duration. She has no history of trauma. Radiographic evaluation reveals compression fractures of T11 and T12. A complete blood count, erythrocyte sedimentation rate, serum protein, serum calcium, phosphate, and parathyroid hormone levels are all within normal ranges. In addition to ordering a dual-energy x-ray absorptiometry (DEXA) scan, which of the following laboratory evaluations is most helpful in evaluating this patient for secondary causes of this presentation?

A bone biopsy
B rheumatoid factor
C serum magnesium
D 25-hydroxyvitamin D
E antinuclear antibodies test

25-hydroxyvitamin D

This patient has typical findings associated with osteoporosis. Most patients with osteoporosis are asymptomatic until fractures present. Fractures occur spontaneously and are associated with back pain of varied degrees. Serum calcium, phosphate, and parathyroid hormone levels are often normal. Since vitamin D deficiency state is common in osteoporosis, a 25-hydroxyvitamin D should be ordered. Bone biopsy is not indicated with this patient; this would be reserved for evaluating for osteomalacia. Rheumatoid factor and antinuclear antibodies would not be of importance with this patient presentation. Serum magnesium is associated more with evaluating for parathyroid or thyroid disorder and has no value with osteoporosis.

A 6-year-old female child presents with complaints of chronic hip pain so severe that she has not been able to walk to the school bus. Examination shows severe tenderness at the left hip with markedly decreased active and passive range of motion. Radiologic examination demonstrates joint effusion with widening. Which of the following is the most likely diagnosis?

A osteochondritis dissecans
B slipped capital femoral epiphysis
C septic hip arthritis
D Legg-Calvé-Perthes disease

Legg-Calvé-Perthes disease
Legg-Calvé-Perthes disease is also known as avascular necrosis of the proximal femur. It typically occurs in children between 4 and 8 years old and persistent hip pain is the main symptom. On examination, the clinician notices a limp and/or limitation of motion of the affected hip. Radiologic examination demonstrates the necrosis with effusion and joint space widening with a negative aspirate. Treatment involves surgical hip replacement. Slipped capital femoral epiphysis (SCFE) is due to the displacement of the proximal femoral epiphysis owing to disruption of the growth plate. The head is normally displaced medially and posteriorly relative to the femoral neck. It typically occurs in adolescence, specifically obese males, and can also be associated with hypothyroidism. SCFE usually occurs after direct trauma to the hip or a fall. Patients complain of vague symptoms at first that progress into pain of the hip or of the knee. On examination, there is decreased internal rotation of the hip that can be confirmed by lateral X-ray of the hip. Septic hip arthritis is not common in children between the age of 5 and 12 years. The legs are held in external rotation to minimize pain and will have a positive aspirate. Osteochondritis dissecans typically presents in the knee, elbow, and talus and is characterized by a wedge-shaped necrosis of bone.

A 78-year-old Caucasian female has a 3-year history of stiffness and achiness of bilateral shoulders and hips. She has been tested for rheumatoid arthritis in the past and has been found negative. Multiple radiographs of her hips and shoulders are unremarkable. She admits that she was placed on prednisone for an allergic reaction and noted a temporary resolution of her symptoms. For the past two weeks she complains of increasing symptoms now involving her neck and pain in her jaw with chewing. Today she noticed that her scalp is sore when she brushed her hair on the right side. What test is the gold standard for diagnosis of this patient's current symptoms?

A ESR 60 mm/h
B temporal artery biopsy
C Elevated CRP
D ultrasound of temporal artery
E polymyalgia rheumatica (PMR) of temporal artery

temporal artery biopsy
The correct answer is (B). This patient has long standing symptoms of polymyalgia rheumatica (PMR) with current symptoms suggestive of giant cell (temporal) arteritis. Temporal artery biopsy is considered the gold standard for diagnosis of giant cell (temporal) arteritis. Patients with temporal arteritis may have an elevated erythrocyte sedimentation rate (ESR) or CRP, but this is not required for diagnosis. A color ultrasound of the temporal artery will sometimes show edema or stenosis of the affected artery but is not very sensitive for giant cell arteritis. MRA is used for diagnosis of larger arteries with vasculitis and not routinely used in the diagnosis of temporal arteritis.

A 64-year-old postmenopausal female presents for an annual examination. She is 5'0" and weighs 92 pounds; compared to her examination 3 years ago she has lost an inch in height. After performing a dual-energy x-ray absorptiometry (DXA) scan the diagnosis of osteoporosis is confirmed and she is started on appropriate treatment. How often should she be recommended to follow-up with a DXA bone density scan?

A Every six months
B Every year
C Every two years
D Every ten years
E No need to follow-up

every two years
Recommendations for patients diagnosed with osteoporosis without a fracture is every 2-3 years (C). This guideline is approved based on age, risk factors, or previous fractures. A post-menopausal woman at age 64 without a presenting fracture can be followed every 2-3 years with bone density imaging (DXA scan). Medications and other treatment options may need earlier or more frequent follow-up depending on the treatment plan. DXA is quite accurate and delivers negligible radiation. Performing a DXA at intervals less than two years has not shown of benefit (A and B). Every 10 years is too far of a time frame to follow osteoporosis (D). Follow-up is needed to see any changes in the bone density (E).

The diagnosis of systemic lupus erythematosus (SLE) is supported by a positive initial antibody screen; however, this test is not specific. Which of the following tests is most specific in the diagnostic evaluation of SLE?

A gliadin antibody
B antibody to double-stranded DNA (anti-dsDNA)
C antinuclear antibody (ANA)
D anticentromere antibody
E antiribosomal P antibody

antibody to double-stranded DNA (anti-dsDNA)
Autoantibody production is the primary immunological abnormality seen in patients with systemic lupus erythematosus (SLE); the antinuclear antibody (ANA) is most characteristic of SLE and seen in 95% of patients with SLE but is not specific for the diagnosis of SLE. A positive ANA can also be found in patients with lupoid hepatitis, scleroderma, rheumatoid arthritis, Sjögren disease, dermatomyositis, and polyarteritis. ANA testing should be employed as the initial screening test in a patient suspected of having SLE. A negative total ANA test is strong evidence against the diagnosis of SLE, whereas a positive test is not confirmatory of the diagnosis. The most specific antibody tests for SLE are antibodies to double-stranded DNA (anti-dsDNAs) and anti-Smith (anti-SM). Although these tests are more specific for SLE, they are less sensitive than the ANA test. Anti-dsDNA is positive in 60% of patients with SLE and anti-SM is positive in 30% of patients. Anti-dsDNA is more likely to reflect disease activity. Gliadin antibody assay is utilized to assess patients with suspected celiac disease. Anticentromere antibody is associated with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome in scleroderma. Antibodies to ribonucleoprotein are present in patients with a mixture of overlapping rheumatological symptoms known as "mixed connective tissue disease."

A 31-year-old female is admitted to the hospital with a microangiopathic hemolytic anemia, thrombocytopenia, and elevated reticulocyte count and serum lactate dehydrogenase. Haptoglobin is decreased and a direct Coombs test is negative. There is a marked increase in schistocytes on blood smear. PT and aPTT are normal. What is the best first line of treatment?

A Aspirin
B Glucocorticoids
C Plasma Exchange
D Platelet transfusion
E Vincristine

plasma exchange
This patient has TTP (thrombotic thrombocytopenic purpura). The mainstay of therapy is plasma exchange. Glucocorticoids are used, but their efficacy has not been demonstrated conclusively. Aspirin is controversial, but has been used in additional therapy. Platelet transfusion is contraindicated. Vincristine is used if plasma exchange does not work.

A 41-year-old male appears pale and mildly icteric. He complains of increasing fatigue lasting several weeks. Laboratory findings note an elevated MCV of 133. What other physical finding would most support a diagnosis of megaloblastic anemia?

A Anorexia
B Dementia
C Diarrhea
D Glossitis
E Paresthesias

Paresthesias
While all choices may be clinical findings in a vitamin B12 deficiency, paresthesias most supports the diagnosis. Peripheral nerves are usually affected first and patients initially complain of paresthesias. The other findings are later in the disease process or appear with very advanced anemia.

A 3-year-old male on laboratory examination has a hemoglobin of 6 (9.5 to 15.0), platelet count of 43,000 (150,000 to 450,000), and Leukocyte count of 9.6 (4.5 to 11.0). Blasts are noted in peripheral smear and on marrow examination. What other laboratory examination is essential in this child?

A Cerebrospinal fluid examination
B Coagulopathy studies
C MRI of the chest
D Skeletal roentgenography
E Urinalysis

CSF
This patient has acute lymphoblastic Leukemia (ALL). Examination of the CSF is essential to rule in/out CNS leukemia. A chest x-ray is adequate to determine if there is enlargement of the thymus or mediastinal nodes. An MRI would not be indicated at this point. Severe bleeds is uncommon and bleeding times are not typically effected. Skeletal roentgenography is not necessary for management of this patient, even if abnormalities are detected. A urinalysis may show microscopic hematuria and the presence of uric acid crystals, but is not essential in the diagnostic workup of this patient.

A patient presents with clinical features of thrombotic thrombocytopenic purpura. They also have an associated diarrhea positive hemolytic uremic syndrome. Which is the best treatment option for this patient?

A Aspirin and dipyridamole
B Glucocorticoids
C Plasma exchange
D Splenectomy and prostacyclin
E Supportive therapy and dialysis

Supportive therapy and dialysis
This patient has a diarrhea-associated hemolytic uremic syndrome with clinical features of TTP. D+HUS is associated with infectious etiologies, usually through food borne illnesses. Treatment consists of supportive therapy, treating the underlying infection, and dialysis if renal function warrants. All other therapies listed are used in the treatment of TTP or diarrhea negative HUS.

A 70-year-old male is being followed for his chronic lymphocytic leukemia. On prior examination, he was staged at Binet Stage A, with three cervical lymph nodes and one right axillary lymph node palpated. Six months later he is seen for follow-up, where he now has five palpable cervical lymph nodes and two left and one right axillary node. His lymphocyte count has risen from 12,500 to 18,300. What is the best therapy at this time?

A Alemtuzumab
B Chlorambucil
C Continue monitoring
D Etoposide
E Fludarabine

C Continue monitoring
This patient continues to remain in Binet Stage A, and therefore would not benefit from beginning any additional therapy. Monitoring should be continued. If they would develop 3/5 lymphoid regions of involvement (cervical, axillary, ileofemoral, splenomegaly, or hepatomegaly) or doubling of the lymphocyte count in less than six months, then adding additional therapy would be indicated. Fludarabine is first-line therapy for progressing CLL. Chlorambucil is the main alkylating agent used in CLL. Alemtuzumab is a monoclonal antibody specific for human CD52, found on most lymphocytes and useful in CLL treatment. Etoposide is used in patients who failed alkylator-based chemotherapy.

A 54 year old male is undergoing treatment for chronic myelogenous leukemia. On examination today his WBC is increased from 17,000 to 67,000, Hgb decreased from 13 to 9, spleen is now palpable on examination and he complains of abdominal fullness, an increased number of marrow blasts are noted on bone marrow biopsy and he "just doesn't feel good". What is the most likely diagnosis?

A Accelerated phase of chronic myelogenous leukemia
B Blast phase of chronic myelogenous leukemia
C Development of treatment related lymphoma
D Molecular transformation phase of chronic myelogenous leukemia
E Transformation of chronic myelogenous leukemia to Acute myelogenous leukemia

Accelerated phase of chronic myelogenous leukemia
This patient has classic signs of transformation from the chronic phase of CML to the accelerated phase. Objective findings to confirm this are increased percentages of blood blasts, thrombocytopenia development, increased basophils, and new clonal cytogenetics abnormalities. Blast crisis is the most severe form of accelerated phase and would indicate the disease is transformed to overt acute leukemia. There is not a phase described as molecular transformation, and the findings are not consistent with a secondary malignancy in this patient.

A 10-year-old male patient has been seen in the clinic on several occasions, with complaints of fatigue and fevers of unknown origin. On prior CBC, he was noted to have a hypereosinophilia. Today's smear reveals multiple small lymphoblasts, with scanty, light-blue cytoplasm after being stained with Wright-Giemsa, and inconspicuous nucleoli. Initial therapy for the patient should include which of the following?

A Bone marrow biopsy and repeat CBC
B Hematologic support for thrombocytopenia
C Immediate referral for radiation therapy
D Placement of an indwelling catheter and IV fluids
E Prevention of metabolic and infectious complications

Prevention of metabolic and infectious complications are the immediate therapies of choice in this patient with ALL. This is done to prevent complications of hyperuricemia, hyperphosphatemia, and infection. Bone marrow biopsy, while important, is not a therapy. Platelet transfusions are only used if there are signs of bleeding and a low platelet count (usually less than 20,000). There is no place for radiation therapy at this point in the patient's initial treatment. Placement of an indwelling catheter is important early in treatment, but is not necessary as an initial treatment.

A 72-year-old male is found to have an absolute lymphocyte count of 13,500 (1000 to 3500). Marrow examination demonstrates infiltration with leukemic lymphocytes, with a lacy or interstitial pattern. What is the most common physical finding that would be associated with this patient?

A Cervical and supraclavicular lymphadenopathy
B Proptosis and headache
C Rhinitis and polyneuropathy
D Splenomegaly and hepatomegaly
E Weight loss and night sweats

Cervical and supraclavicular lymphadenopathy
All of these findings can be present in this patient with chronic lymphocytic leukemia, but the most common physical finding in these patients are lymphadenopathy with over 80% presenting with nontender adenopathy. All others are findings that may occur, but are significantly less common

A patient being treated for Burkitt's lymphoma is found to have hyperkalemia, hyperphosphatemia, hypocalcemia, a metabolic acidosis, hyperuricemia, and appears to be going into renal failure after the first dose of chemotherapy. To prevent or minimize this finding, the best therapy is to start which of the following?

A Allopurinol and rasburicase
B Colchicine and monitored hydration of at least 3 L/day
C Continuous venovenous hemofiltration and colchicine
D Continuous venovenous hemofiltration and hydration
E Monitored hydration of at least 3 L/day and allopurinol

Monitored hydration of at least 3 L/day and allopurinol
This patient has developed a tumor lysis syndrome. The usual prophylactic therapy is carefully monitored hydration of at least 3 L of saline per day, and either allopurinol or rasburicase to decrease serum uric acid concentration and hyperuricosuria. Allopurinol and rasburicase alone are not enough to prevent TLS. Colchicine is a gout medication and not appropriate in this setting. Continuous venovenous hemofiltration is useful in allowing concomitant full-dose chemotherapy and preventing tumor lysis and renal failure, but is not the preventative treatment prior to starting chemotherapy.

A 74-year-old male has been diagnosed with aplastic anemia. His temperature is 37.4°C, heart rate is 68, respiratory rate is 20, and blood pressure is 130/86. Mild petechiae are noted to the extremities and there is some scattered bruising. The patient is currently taking Captopril, HCTZ, Lipitor, and Flo-max. Laboratory findings include:
WBC: 2.9
HGB: 10.4

PLT: 54,000

Initial management for this patient should include which of the following?

A Broad spectrum antibiotics
B Discontinuation of Captopril
C Immediate assessment for allogeneic stem cell transplant
D Irradiated and leukocyte depleted red cell transfusion
E Platelet transfusion

discontinuation of captopril
While the laboratory findings demonstrate a pancytopenia, levels are not low enough or physical findings are not suggestive of the need for transfusions at this time. Given the patients age, he is not eligible for an allogeneic stem cell transplant. He has no physical findings suggestive of a systemic infection requiring antibiotics. Captopril has a low risk of causing pancytopenias. It is best to discontinue this drug first as a possible cause of the pancytopenia, while continue to treat this patient symptomatically.

A 68-year-old female has been having increasing low back pain that radiates into her right hip and down her right leg. She has failed physical therapy and anti-inflammatories. She feels like the bones "are rubbing on each other." Radiographic imaging of the spinal cord, hip, and leg reveal several lytic lesions. The most helpful finding to confirm the diagnosis would include which of the following?

A Reed-Sternberg cells in the marrow
B Granulocytosis in the marrow
C Macrocytosis in the peripheral smear
D Monocytosis in the peripheral smear
E Plasmacytosis in the marrow

Plasmacytosis in the marrow
Plasmacytosis in the marrow is the most prominent and diagnostically important finding. Reed-Sternberg cells are seen in Hodgkin's lymphoma. Granulocytosis is an increased white cell count seen in leukemias. Macrocytosis is enlargement of red cells, and can be seen in vitamin B12 deficiency. Monocytosis is an increase in monocytes, and can be seen in infectious mononucleosis or some leukemias.

A patient is undergoing induction treatment for acute myelogenous leukemia. They received daunorubicin and cytarabine seven days ago. They present in the emergency department with a temperature of 38.4°C, HR: 90, RR: 20. They are complaining of cough, fatigue, and diarrhea. They appear pale, fatigued, and slightly tachypenic. Lung fields are clear to auscultation interrupted by cough, and bowel sounds are hyperactive. What is the next best step in caring for this patient?

A Obtain blood cultures and start cefepime
B Obtain chest x-ray and blood cultures
C Obtain CBC, LFTs, and blood cultures
D Obtain CT of the chest and admit
E Obtain pulse ox and start O2 by nasal cannula

Obtain blood cultures and start cefepime
This patient has developed a neutropenic fever following induction chemotherapy. The most important next step is to obtain blood cultures and start IV antibiotics. Additional steps can include urine and sputum culture. Consideration should be given for both gram positive and negative coverage. The lungs are clear due to the inability to form pus with neutropenia. A chest x-ray or CT may be appropriate, but are not the next best steps. Currently, there is no reason to start oxygen and this would not be the next best step.

A 41-year-old alcoholic male, who lives primarily on the streets, appears pale, cachectic, and mildly icteric. He is complaining of several weeks of increasing fatigue. Laboratory findings note an elevated MCV of 128. What other physical finding would most support the diagnosis for megaloblastic anemia?

A Decreased vibration and position sense
B Dementia
C Difficulty with balance
D Glossitis
E Parethesias

Glossitis
Features of folate deficiency are similar to vitamin B12 deficiency. However, there are none of the neurologic abnormalities associated with vitamin B12. Glossitis is the only non-neurologic finding in the PE that would support folate deficiency. Alcoholism and poor dietary intake also support the diagnosis of folate deficiency.

A 56-year-old chronic alcoholic presents with signs of anemia. Laboratories reveal a moderate anemia with a hematocrit of 30% (45 to 62%) and a dimorphic population of red cells, one normal and one hypochromic. The MCV (mean corpuscular volume) is normal, the serum iron level is elevated, and the transferrin saturation is high. The diagnosis was made using which diagnostic study?

A 24-hour urine for porphyrin
B Bone marrow biopsy
C Echocardiogram
D Ferritin level
E Liver biopsy

Bone marrow biopsy
Diagnosis of sideroblastic anemia is made by examination of the bone marrow, using Prussian blue staining and noting the presence of ringed sideroblasts, which are cells with iron deposits encircling the red cell nucleus. None of the other studies are useful in making this diagnosis.

A newborn male is diagnosed with Christmas factor deficiency. What is the likelihood that he inherited this disorder from his father?

A 0%
B 25%
C 50%
D 75%
E 100%

0%
All daughters of a hemophilic male are carriers of hemophilia, whereas all sons are normal. Hemophilia B (or Christmas factor deficiency) is one of only two sex-linked pattern-bleeding disorders, and as such the disease occurs almost exclusively in males. Sons of carriers have a 50% chance of being affected and daughters of carriers have a 50% chance of being carriers themselves.

A 14-month-old African American boy is diagnosed with sickle cell anemia. Neither parent has the disease. If you are counseling the parents of this child on the likelihood of another one of their children having sickle cell disease, what percentage would you tell them?

A 0%
B 25%
C 50%
D 75%
E 100%

25%
S s
S SS Ss
s Ss ss
Sickle cell anemia is an autosomal recessive genetic disorder. The presence of two defective genes is required for sickle cell anemia to occur. If each parent carries one sickle hemoglobin gene and one normal gene, each child has a 25% chance of inheriting two defective genes and developing sickle cell, a 25% chance of inheriting two normal genes and not having the disease, and a 50% chance of being an unaffected carrier.

A 27-year-old female presents to the emergency department with severe RUQ pain. Ultrasonography reveals gallstones. In preparation for a potential cholecystectomy, a CBC is obtained that reveals a normocytic anemia with an increased mean corpuscular hemoglobin concentration (MCHC). She is slightly jaundiced and you are able to palpate her spleen on examination. What is her underlying diagnosis?

A Hereditary spherocytosis
B Iron deficiency anemia
C Sickle cell anemia
D Thalassemia
E Von Willebrand's disease

hereditary spherocytosis
Hereditary Spherocytosis (HS) is characterized by jaundice, an enlarged spleen, and often gallstones; gallstones are more frequently seen in young people, triggering the investigation into HS. An increased MCHC is a characteristic feature of HS and is almost the only condition in which this finding is seen. Iron deficiency anemia does not have an increased MCHC. Sickle Cell has characteristic findings different than the presenting findings. Thalassemia is noted for a microcytosis and Von Willebrand's is a coagulation disorder.

In a 73-year-old male, laboratory and radiologic findings that demonstrate marrow plasmacytosis with >30% plasma cells, a monoclonal globulin spike present, and lytic bone lesions is most consistent with which of the following?

A Acute myelogenous leukemia
B Chronic myelogenous leukemia
C Hodgkin lymphoma
D Multiple myeloma
E Non-Hodgkin lymphoma

Multiple myeloma
The diagnosis of plasma cell myeloma is confirmed when at least one major and one minor criterion, or three minor criteria, are documented in a patient. This patient has one major and two minor criteria. AML and CML are not associated with lytic lesions. Hodgkin and Non-Hodgkin lymphoma typically do not have lytic lesions or monoclonal globulin spikes.

A 31-year-old female presents with headache, fever, and petechiae to her lower extremities. A microangiopathic hemolytic anemia is noted. She denies diarrhea, recent infectious episodes, cough, shortness of breath, or urinary symptoms. What other finding would you expect to find in this woman to confirm the diagnosis?

A Blasts on blood smear
B Edema
C Hepatomegaly
D Thrombocytopenia
E Sickle Cell

thrombocytopenia
This patient has classic signs of thrombotic thrombocytopenic purpura. Microangiopathic hemolytic anemia and thrombocytopenia are seen in all cases of TTP in the absence of another plausible explanation. Edema is not seen in TTP. Sickle cell is found in sickle cell anemia. Hepatomegaly is seen in less than 20% of cases of TTP and does not confirm the diagnosis. Blasts are seen in leukemia and do not confirm the diagnosis.

A central component of hemostasis, it also functions as a carrier factor and an adhesive link between platelets and injured blood vessel walls. Dysfunction of this factor is the most common inherited bleeding disorder in humans. Which factor has these characteristics?

A Factor VII
B Factor VIII
C G6PD deficiency
D Tumor necrosis factor
E Von Willebrand's factor

Von Willebrand Factor
Von Willebrand's factor is a central component in hemostasis, and is a carrier for factor VIII as well as an adhesive molecule. It is the most common bleeding disorder in the general population. G6PD deficiency causes anemia. Tissue necrosis factor plays a role in the regulation of immune cells. Factor VII and VIII deficiencies cause coagulation disorders, but are not the most common.

A 38-year-old male presents with red-brown urine in the mornings. The discoloration has come and gone several times over the last several months. He has also noted hard, painful areas on his skin, which seem to be located over veins, which have occurred intermittently. Laboratories reveal nonspherocytic red cells and a Coombs negative intravascular hemolysis. What is the most likely diagnosis?

A Acute Myelogenous Leukemia
B Aplastic Anemia
C Iron Deficiency Anemia
D Paroxysmal Nocturnal Hemoglobinuria
E Von Willebrand's Disease

Paroxysmal Nocturnal Hemoglobinuria
Urine that appears to be bloody, primarily in the morning and episodic in nature, along with thrombosis of dermal veins, nonspherocytic red cells and Coombs negative intravascular hemolysis are classic signs of PNH. PNH may be a symptom of aplastic anemia, but this patient doesn't have a pancytopenia. He also does not have a microcytic anemia, which rules out iron deficiency. Von Willebrand's is a coagulation disorder.

A 54-year-old male complains of easy fatigability, loss of sense of wellbeing, anorexia, weight loss, and excessive sweating that has been going for approximately three months. On physical exam he has pallor and splenomegaly. White count is 98,000 (4 to 12,000). The marrow is hypercellular and contains the Ph chromosome. What is the most likely diagnosis?

A Acute lymphocytic leukemia
B Acute myelogenous leukemia
C Chronic lymphoblastic leukemia
D Chronic myelogenous leukemia
E Multiple Myeloma

CML
This patient has chronic myelogenous leukemia. 90% of patients presenting with CML are Ph chromosomes positive, and have vague symptoms and complaints that have been going on for weeks to months. Acute lymphocytic leukemia on CBC tends to have an eosinophilia. Acute myelogenous leukemia is not associated with the Ph chromosome. Chronic lymphoblastic leukemia marrow examination notes lymphocytic infiltrates. Multiple myeloma is noted for light chains and not ph chromosome.

A 27-year-old male with enlarged cervical lymph nodes and shortness of breath is seen. Lungs are clear to auscultation but diminished breath sounds centrally are noted. A chest x-ray reveals a large mediastinal mass. A biopsy of the lymph node is performed and Reed-Sternberg cells are noted on exam. What is the most likely diagnosis?

A Acute myelogenous leukemia
B Chronic lymphocytic leukemia
C Hodgkin lymphoma
D Infectious mononucleosis
E Multiple myeloma

Hodgkin lymphoma
The presence of Reed-Sternberg cells in the histologic examination of lymph nodes, along with a large mediastinal mass, is consistent with Hodgkin lymphoma. Reed-Sternberg cells are not seen in multiple myeloma, AML, CLL, or infectious mononucleosis.

Deficiency of what factor may predispose a person to recurrent thrombosis?

Answer Choices
1 Platelet deficiency
2 Factor VIIIC deficiency
3 Protein C deficiency
4 von Willebrand factor deficiency
5 Factor VII deficiency

Protein C deficiency
Explanation
Deficiency of pro-coagulants causes bleeding diathesis while deficiency of anti-coagulants causes recurrent episodes of thrombosis.

Protein C is a potent anti-coagulant. Both protein C and co-factor protein S are vitamin K dependent peptides. Protein C, which is produced by the liver, is a powerful naturally occurring anti-coagulant and is activated by thrombin. Thrombin (a powerful pro-coagulant) activates protein C after binding with a factor known as thrombomodulin. Activated protein C digests factor VIIIA and Va. Protein S is produced by endothelial cells and acts as a co-factor for protein C.

A 2-year-old boy presents for routine check up. He is a known case of sickle cell disease and is on penicillin prophylaxis. The child is asymptomatic and appears healthy. He has received a regular schedule of immunizations to date. What additional vaccination needs to be administered in his case?

Answer Choices
1 Influenza, pneumococcal, and meningococcal vaccine
2 DTaP and Hepatitis B vaccine
3 IPV and varicella vaccine
4 Tdap, MMR, and Hepatitis B vaccine
5 Only influenza vaccine

Influenza, pneumococcal, and meningococcal vaccine
Explanation
Children with sickle cell disease require influenza, pneumococcal, and meningococcal vaccine immunization, in addition to the regular childhood vaccination.

Heptavalent pneumococcal conjugate vaccine (PCV) should be administered to all children at 2, 4, and 6 months and a booster at 12 - 15 months of age. In addition to this, children with sickle cell disease require another type of pneumococcal vaccine called the 23-valent pneumococcal vaccine (PPV) that needs to be administered between 2 - 5 years of age, as well as a booster at 5 years. The PPV vaccine is recommended in addition to PCV in certain high risk groups who have a higher risk of developing pneumococcal disease. These include sickle cell disease, functional or anatomic asplenia, immunocompromised individuals, and chronic cardiac or pulmonary disease.

Influenza vaccine is recommended for children between 6 - 23 months of age as routine immunization, whereas certain high risk groups such as sickle cell disease, diabetes, HIV, cardiac disease, and conditions that compromise the respiratory function require annual influenza vaccination.

Meningococcal conjugate vaccine is administered to all children aged 11 - 12 years, as well as unvaccinated adolescents at 15 years of age and to college freshmen living in dormitories. Vaccination is also recommended to children above 2 years with high risk, such as complement deficiency, sickle cell disease, and persons with functional or anatomic asplenia.

DTaP, Hepatitis B vaccine, IPV, varicella vaccine, Tdap, and MMR vaccine are not the additional vaccines required in sickle cell disease.

A 45-year-old Caucasian man is employed to demolish numerous painted structural steel railroad trestles. The project is scheduled to take 2 years. He is not given protective masks or clothing. Towards the end of the project, he starts to develop abdominal pain, so he sees his family doctor. On questioning during the medical history, he comments that he frequently drops things with his right hand. On physical examination, his lungs are clear to auscultation and no abnormal breath sounds are heard. His cardiac exam is unremarkable. His skin exam is normal other than a pigmentation at the gingivodental margin. His extensor muscles of the wrist and finger are weak. A CBC is done; the results are shown in the chart.

TEST RESULTS REFERENCE RANGE
RBC 4.5 x 106/µl 4.5 - 5.7 x 106/µl (male) 3.9 - 5.0 x 106/µl (female)
Mean corpuscular volume 73 cu µ 80 - 100 cu µ
Hematocrit 30 40 - 50 (male) 36 - 44 (female)
Hemoglobin 10 gm/dL 13.8 - 17.2 gm/dL (male)
12.1 - 15.1 gm/dL (female)
Platelets 250,000/mm3 130,000 - 400,000 mm3
Comments: Red blood cell stippling is present. Hypochromic red blood cells are present.

Question
Where is the toxic element stored?

Answer Choices
1 Bones
2 Red blood cells
3 Liver
4 Skin
5 Brain

Bone
Explanation
This patient has an occupational history, signs, and symptoms consistent with lead poisoning. Abdominal pain, red blood cell basophilic stippling, wrist drop, and a gingival lead line are all consistent with lead poisoning. Anemia can also be seen with lead poisoning. The anemia can be either normocytic normochromic or microcytic hypochromic, as is the case here.

Bones are the primary location in the body where lead is stored.

Lead can affect the brain, causing encephalopathy. It affects the red blood cells, causing anemia and red blood cell basophilic stippling. It affects the skin; a "lead line" can be seen in the gingivodental margin. It can affect the kidney, resulting in Fanconi's syndrome. However, bones are the primary sites in the body where lead will be stored.

A 22-year-old African-American man presents with excruciating pain in his back for the past 2 days. He denies any history of trauma. He gives a history of similar pains in the past; however, he is not on any medications. His older sister has a similar illness. On examination, his HR is 110 beats/minute, and he has a tinge of jaundice.

Question
What laboratory result would you expect after analyzing his blood?

Answer Choices
1 Hemoglobin of 16 g/dl
2 Hematocrit of 21%
3 Microcytic red cells
4 Reticulocytosis of 2%
5 Bilirubin of 1 mg/dl

Hematocrit 21%
Explanation
The correct response is hematocrit of 21%. The clinical picture is suggestive of a vaso-occlusive painful crisis in a patient with sickle cell disease.

Sickle cell disease is an inherited hemolytic anemia occurring primarily in persons of African descent. In Hemoglobin S, valine replaces glutamic acid as the 6th amino acid on the beta hemoglobin chain. Homozygous inheritance of hemoglobin S (HbSS) results in sickle cell anemia. Heterozygotes (Hb AS) have the sickle cell trait and do not experience vaso-occlusive crisis.

The diagnosis is confirmed by hemoglobin electrophoresis. Homozygotes have Hb S with a variable amount of Hb F. Heterozygotes have more Hb A than Hb S.

The hemoglobin may range from 5 to 10 g/dl (hematocrit of 15 to 30%) in sickle cell anemia; the red blood cell count typically ranges from 2 and 3 million/mL. The peripheral blood film reveals normocytic red cells. Reticulocytosis of 10% to 20% is a common finding. The mean cell volume (MCV) may be slightly elevated due to the increased reticulocyte count. Leukocytosis may be present as a result of a precipitating bacterial infection or the crisis itself. As it is a chronic hemolytic state, serum bilirubin levels are usually elevated, with values of 2 to 4 mg/dl or even higher in severe jaundice. Fecal and urinary urobilinogen levels are also elevated.

A 67-year-old man is recovering following an uncomplicated total hip arthroplasty. His past medical history is significant for hypertension and mild asthma. On postoperative day 2, he begins to note stiffness in his right leg. On exam, his right calf is noted to be swollen and slightly warmer than the left leg. The remainder of the exam is unremarkable. He is afebrile, and his vital signs are stable.

Question
What diagnostic evaluation is most appropriate in this situation?

Answer Choices
1 Compression ultrasonography
2 Contrast venography
3 Contrast-enhanced MRI
4 Lower extremity X-ray
5 Serum B-type natriuretic peptide (BNP) measurement

Which of the following electrolyte imbalances occurs most frequently in patients with cancer?
Answer Choices
1 Hypokalemia
2 Hyponatremia
3 Hypercalcemia
4 Hypomagnesemia
5 Hypocalcemia

hypercalcemia
Explanation The correct answer is hypercalcemia. Hypercalcemia occurs most frequently in patients with cancer, particularly those with lung, breast, multiple myeloma, or bone mestastases.
Hyponatremia, hypomagnesemia, and hypocalcemia are all incorrect. They can all manifest at some point during therapy, but hypercalcemia is the most frequently noted complication.

A 30-year-old Caucasian man presents with a prolonged bleeding time. The platelet count is normal, and there is no history that suggests an apparent cause of acquired platelet dysfunction (e.g., drugs). If the ristocetin cofactor activity is decreased, what is the most likely diagnosis?

Answer Choices
1 Idiopathic thrombocytopenic purpura
2 von Willebrand disease
3 Hemolytic-uremic syndrome
4 Hypersplenism
5 DIC

von Willebrand disease
Explanation
Primary hemostasis in response to vessel injury is achieved as platelets adhere to the subendothelial matrix in a reaction that requires von Willebrand factor (vWF). vWF is synthesized by endothelial cells and megakaryocytes and forms multimers containing from 2 to more then 40 subunits. vWF is required for normal platelet adhesion and prolongs the half-life of Factor VIII.

Ristocetin cofactor activity is determined by the ability of ristocetin to enable vWF to interact with platelet glycoprotein Ib in vitro. It is reduced in most patients with von Willebrand disease but may also be reduced when there is an elevated level of plasma protein, such as paraprotein. Ristocetin-induced platelet agglutination is decreased in most types of von Willebrand disease except type 2B, in which agglutination occurs in very low concentrations of ristocetin.

vWF antigen (factor VIII-related antigen) can be measured by various immunoassays, and the size distribution of vWF multimers can be determined by crossed-immunoelectrophoresis on agarose gel. These tests are useful for subclassification of von Willebrand disease.

Thrombocytopenia is characteristic for idiopathic thrombocytopenic purpura, hemolytic-uremic syndrome, hypersplenism, and DIC.

A 44-year-old woman presents for her annual exam. Findings at the physical exam are BP-120/80 mmHg, HR - 70 beats/min, pale mucous membranes, and increased uterine size. The patient also presents with menorrhagia of 2 years duration. Her blood test shows microcytic hypochromic red blood cells (RBCs). Further exams show low serum iron, increased total iron-binding capacity (TIBC), and low ferritin. What is the most appropriate diagnosis for this patient?

Answer Choices
1 Anemia of chronic disease
2 Anemia secondary to iron deficiency
3 Beta thalassemia
4 Megaloblastic anemia
5 Sideroblastic anemia

Anemia secondary to iron deficiency
Explanation
The clinical and laboratory findings are suggestive of anemia due to iron deficiency. Low serum iron, increased total iron-binding capacity (TIBC), and low ferritin along with the patient's typical peripheral smear of microcytic hypochromic red blood cells are all findings supportive of a diagnosis of anemia secondary to iron deficiency. The most common causes of iron deficiency are deficit in the diet and blood loss from conditions such as menorrhagia. The diagnostic tests are a blood smear showing microcytic hypochromic red blood cells. The serum iron concentration is low. Transferrin or total iron-binding capacity (TIBC), which is the protein that transports iron, is elevated, but the saturation of this protein is decreased. Ferritin, which is the protein that stores iron in the body, is low due to excessive depletion of the iron stores. In addition to the administration of iron and a high iron-bioavailability diet, treatment depends on the cause of the bleeding.

Low serum iron, low TIBC, and increased ferritin are the lab findings seen in anemia due to chronic disease or inflammation. It can be seen as a result of infections, inflammatory diseases, or neoplasias. Blood smear can be normal or can show similar characteristics to the one seen in iron deficiency. The serum iron is low and the TIBC is low with low percentage saturation; however, the serum ferritin levels are high. The patient has good storage of iron, but it cannot be used in the production of red cells. The treatment of this type or anemia consists of treating the underlying disease.

Normal-to-increased serum iron levels, normal TIBC, and increased ferritin are characteristic of sideroblastic anemia. This group of anemia is characterized by erythroblasts in bone marrow showing iron deposits in their mitochondria on Prussian blue staining. The etiology can be idiopathic, hereditary, or toxic, such as in cases of lead intoxication. Blood smear can be normal or show microcytic hypochromic red cells. The treatment of this type of anemia consists of treating the underlying cause, pyridoxine, iron chelation with desferrioxamine, and blood cells transfusions.

Megaloblastic anemia is a condition in which the red blood cells are bigger than normal (MCV >115 fl). There are many different causes, such as drugs (anticonvulsants, methotrexate, etc.), but deficiencies of vitamin B12 and folic acid in the diet are the most common cause. Megaloblastic anemia due to nutritional deficiency of vitamin B12 takes years to manifest, because the reserve is used very slowly. Patients experience features of anemia and neurological symptoms, such as confusion and peripheral neuropathy. Folic acid deficiency in the diet may also produce megaloblastic anemia, but it does not produce neurological symptoms.

Thalassemias are a group of inherited disorders; they are characterized by a decrease in the production of the alpha or beta chains of hemoglobin. The diagnosis is done by genetic studies, and there is no definitive treatment available yet.

A 10-month-old African-American infant presents with a recent onset of symptoms suggestive of sickle cell anemia. He is the younger of 2 children, and his mother is concerned about his health because of the symptoms and a family history of sickle cell anemia.

Question
What are the 1st symptoms usually seen in these children?

Answer Choices
1 Retinal deterioration
2 Yellowing of skin and eyes
3 Delayed growth
4 Tachycardia and fever
5 Painful swelling of hands and feet

Painful swelling of hands and feet
Explanation
The correct response is painful swelling of hands and feet.

Patients with sickle cell anemia (SCA) are often asymptomatic during the 1st few months of life. When patients are over 6 months of age, the 1st symptoms usually make up what is known as hand-foot syndrome (dactylitis). The etiology is vasoocclusion and can be triggered by dehydration, cold temperatures, stress, or infection. Pain management is the hallmark of treatment for children with these symptoms.

Patients with SCA can experience retinopathy that is often silent until very serious complications occur (e.g., retinal detachment, hyphema). While retinal deterioration is something that does occur with SCA, it would not be a presenting symptom.

The yellowing of skin and eyes are signs of jaundice; this can occur in patients with SCA due to the rapidly occurring breakdown of red blood cells. These symptoms are usually not present at the onset of the symptoms.

Both delayed growth and delayed puberty are symptoms of SCA, but are long-term sequelae and would not be the precipitating factor for an initial office visit for a diagnosis of SCA.

Tachycardia and fever are not specific, and they could be related to various illnesses; however, when related to SCA, tachycardia and fever may be associated with an infection or acute chest syndrome. Bilateral infiltrates caused by a decrease in hemoglobin and pulmonary infarction can also be seen in acute chest syndrome. Acute chest syndrome is the most common cause of death in patients with SCA. Infection was more common in the past, but has become more controlled and less of an issue; it is still prevalent in patients with SCA.

A 70-year-old man presents for a follow up with his cardiologist. There are no specific complaints. Findings at the physical exam are BP 130/80 mmHg, HR 80 beats/min, and the appearance of pale mucous membranes. Lungs are clear to auscultation, and there is no edema of lower extremities. Fecal occult blood test (FOBT) was negative. Blood test shows hypochromic microcytic RBCs. Further exams show low serum iron, low total iron-binding capacity (TIBC), and increased ferritin. What is the most likely diagnosis?

Answer Choices
1 Anemia of chronic disease
2 Anemia secondary to iron deficiency
3 Beta thalassemia
4 Megaloblastic anemia
5 Sideroblastic anemia

Anemia of chronic disease
Explanation
The lab findings of low serum iron, low total iron-binding capacity (TIBC), and increased ferritin are suggestive of chronic disease or inflammation as the cause of the patient's anemia. It can be seen as a result of infections, chronic inflammatory diseases, or neoplasias. Blood smear can be normal or can show similar characteristics to those seen in iron deficiency anemia. The serum iron is low and TIBC is low, with low percentage saturation; however, the serum ferritin level is high. The patient has good storage of iron, but it cannot be used in the production of red cells. The treatment of this type of anemia consists of treating the underlying disease.

Low serum iron, increased TIBC, and low ferritin are seen in patients with microcytic anemia secondary to iron deficiency. The most common causes of iron deficiency are blood loss and deficit in the diet. In developed countries, a dietary deficiency of iron is rare. Diagnostic test includes a blood smear showing microcytic hypochromic RBCs. The transferrin or total iron-binding capacity (TIBC), which is the protein that transports the iron, is elevated, but the saturation of this protein is decreased. Ferritin, which is the protein that stores iron in the body, is low due to excessive depletion of the iron stores. In addition to the administration of iron and a high iron-bioavailability diet, treatment depends on the cause of the bleeding.

Normal-to-increased serum iron levels, normal TIBC, and increased ferritin are characteristic of sideroblastic anemia. This is characterized by erythroblasts in bone marrow showing iron deposits in their mitochondria on Prussian blue staining. The etiology can be idiopathic, hereditary, or toxic, such as in cases of lead intoxication. Blood smear can be normal or show microcytic hypochromic red cells. The serum iron is increased, the TIBC is normal, and the ferritin is increased. The treatment of this type of anemia consists of treating the underlying cause through administration of pyridoxine, iron chelation with desferrioxamine, and blood cells transfusions.

Megaloblastic anemia is a condition in which the red blood cells are bigger than normal (MCV >115 fl). There are many different causes, such as drugs (anticonvulsants, methotrexate, etc.), but deficiencies of vitamin B12 and folic acid in the diet are the most common cause. Vitamin B12 deficiency takes years to manifest because the reserve is used very slowly. Patients experience anemia and neurological symptoms, such as confusion and peripheral neuropathy. Folic acid deficiency produces megaloblastic anemia, but it does not produce neurological symptoms.

Thalassemias are a group of inherited disorders; they are characterized by a decrease in the production of the alpha or beta chains of hemoglobin. The diagnosis is done by genetic studies, and there is no definitive treatment available yet.

A 77-year-old man presents with left-sided chest pain, headaches, night sweats, a burning sensation in his hands and feet, and shortness of breath. 2 days ago, he began having headaches; his symptoms have progressively worsened.

On exam, the patient is a thin, ill-appearing man. His spleen is palpated 8 cm below the costal margin and the liver 5 cm below the costal margin. Laboratory analysis shows that his red blood cell mass is elevated, and he has a decrease in plasma volume. White blood cells and platelets are within normal limits. Appearance of the red blood cells is normal. Arterial blood gas analysis shows an O2 saturation of 94% on room air.

Question
What is the most likely cause of this patient's symptoms?

Answer Choices
1 Chronic myelogenous leukemia
2 Polycythemia vera
3 Thrombocytopenia
4 Iron-deficiency anemia
5 Aplastic anemia

Polycythemia vera
Explanation
Polycythemia vera occurs when there is an abnormal increase in red blood cells due to an increase in production by the bone marrow. It may be accompanied by an increase in white blood cells and platelets, as well. It occurs more frequently in men than in women, at an average age of 60.

Patients with polycythemia vera may remain symptom-free for years. When symptoms do occur, they can include weakness, fatigue, headache, light-headedness, shortness of breath, vision problems, night sweats, bleeding gums, itchy skin that worsens after bathing, and a burning sensation in the hands and feet. Symptoms are usually due to the increase in the blood's thickness and altered clotting.

Diagnosis is made by bone marrow biopsy, complete blood count with differential, vitamin B12 level, hematocrit, and blood volume.

Treatment involves reducing the thickness of the blood. This can be done by phlebotomy or chemotherapy. Chemotherapy involves using hydroxyurea to suppress the bone marrow. Interferon may be used to lower blood cell counts, and anegrelide may lower the number of platelets.

This patient does not have chronic myelogenous leukemia; his white cell count is normal.

Thrombocytopenia is not likely. The number of platelets is normal.

Iron-deficiency anemia is not likely. The patient's red blood cells are of normal size and shape.

Aplastic anemia is not likely. The patient's white blood cell and platelet numbers are normal.

A 17-year-old boy presents with abdominal pain following a motor vehicle accident. The patient was the passenger in the vehicle and was wearing restraints. He states that he has pain in the left side of his abdomen, which began at the time of the accident. Physical exam revealed a well-developed young man in moderate distress. He had left upper quadrant tenderness, nausea, and positive Kehr's sign. He also had diaphoresis, tachycardia, and hypotension.

Question
What is the most likely cause of this patient's symptoms?

Answer Choices
1 Ruptured abdominal aorta
2 Hepatic injury
3 Splenic injury
4 Ruptured diaphragm
5 Diverticulitis

splenic injury

The spleen is located in the left upper quadrant of the abdomen and is surrounded by the ribs on 3 sides (laterally, anteriorly, and posteriorly). It is in contact with many organs, including the pancreas, diaphragm, stomach, and kidney/adrenal glands. There are several ligaments attached to it, including the splenocolic, splenorenal, gastrosplenic, and splenophrenic. The spleen is often injured through blunt trauma to the abdomen. This can occur from motor vehicle accidents, gunshot, knife, and crush wounds. Injuries on the surface can result in subcapsular hematomas and capsular lacerations. Injury may also reach the internal structures. Injury often results in hemorrhages, which can be small or large. As a result, the patient can have abdominal pain, a positive Kehr's sign (pain in the tip of the shoulder when a person is lying down and the legs are elevated), distension, and may even go into shock due to massive blood loss.

Diagnosis of splenic injury is usually diagnosed by CT scan or ultrasound. Treatment is usually splenectomy. If there is an underlying infection, then splenectomy should be avoided. If the injury is small enough, the patient may be monitored and may not need surgery.

A 17-year-old boy presents with a 3-week history of severe, worsening fatigue. Physical examination reveals new onset wheezing and abdominal pain. Diagnostic labs reveal severe hyperuricemia and elevated serum lactate dehydrogenase. Based on the history, physical, and other studies, a diagnosis of lymphoblastic lymphoma is made for this patient.
Question
The combination chemotherapy this patient will undergo is based on the regimen for what other malignant process?

Answer Choices
1 Acute lymphoblastic leukemia (ALL)
2 Acute myelogenous leukemia (AML)
3 Chronic lymphoblastic leukemia (CLL)
4 Chronic myelogenous leukemia (CML)
5 Anaplastic large cell lymphoma

ALL
Explanation
The preferred treatment plan for lymphoblastic lymphoma is combination chemotherapy based on regimens used for acute lymphoblastic leukemia (ALL). With this approach, children with early stage (I or II) or advanced stage (III or IV) lymphoblastic lymphoma achieve survival rates of >90 and >80 percent, respectively.

There are currently no regimens to treat lymphoblastic lymphoma with the treatment protocols for the pathologies of AML, CLL, CML, or anaplastic large cell lymphoma.

A 3-year-old African-American girl with sickle cell disease presents with a limp; her mother first noticed the limp yesterday morning. The girl has not had any fever, nausea, or vomiting, but she in pain. The girl's sickle cell disease has been relatively mild; she is fully immunized and takes prophylactic oral penicillin 125 mg 2 times a day.

On exam, she is afebrile. There is pain in the left hip on internal and external rotation, but there is no definite point tenderness. Exam of the left knee and right hip are negative. You are concerned about septic arthritis, and you plan further work up. What organism are you most concerned about?

Answer Choices
1 Staphylococcus aureus
2 Streptococcus species
3 Haemophilus influenzae type b
4 Salmonella species
5 Neisseria gonorrhoeae

Salmonella species
Explanation
The correct response is salmonella species.

Choosing the initial antibiotic therapy in suspected osteomyelitis depends on the likely bacterial pathogens based on the age of the patient, the results of Gram staining of the joint aspirate, and consideration of any special circumstances, such as this patient's sickle cell disease. In otherwise healthy children under age 5, the most likely causative organisms in osteomyelitis are S. aureus, Streptococci, and H. influenzae type b in children that have not been immunized.

However, in children with sickle cell disease, Gram-negative enteric pathogens are also common culprits. In particular, children with sickle cell disease are at risk for Salmonella septicemia and osteomyelitis. Therefore, appropriate empiric antibiotic therapy needs to cover these organisms, pending culture and susceptibility studies, in patients with sickle cell anemia.

A 46-year-old woman presents with generalized petechiae, left-sided weakness, headaches, mild confusion, nausea, and vomiting. She is febrile, with an oral temperature of 103° F. On exam, she appears mildly confused and has decreased motor strength in the left extremities. Petechiae are scattered over the inner thighs and upper extremities.

Significant lab values are as follows: Hgb 7.2 g/dL, Hct 22%, WBC 10.2 x 103/µL, and platelet count 22,000/µL. Peripheral blood smear is remarkable for the presence of schistocytes. Coagulation studies and serum chemistries are unremarkable, with the exception of elevated bilirubin and LDH levels. What is the first-line therapy for her condition?

Answer Choices
1 Prednisone
2 Exchange plasmapheresis
3 Splenectomy
4 Dipyridamole
5 Platelet transfusion

exchange plasmapharesis
Explanation
Thrombotic thrombocytopenic purpura (TTP) is a condition of unknown etiology characterized by thrombocytopenia, microangiopathic hemolytic anemia, and various neurologic abnormalities. Fever and renal abnormalities can also be present to varying degrees. Hyperbilirubinemia is often present because of hemolysis, and the LDH is frequently elevated out of proportion to the hemolysis. TTP is considered a medical emergency; it is initially treated by daily plasmapheresis until a safe platelet count (150,000/µL) is achieved and serum LDH begins to fall. Long-term responses have been achieved in about 90% of patients with this therapy.

Corticosteroids (e.g., prednisone) are often used in conjunction with plasmapheresis, but they are ineffective when used as the sole therapeutic agent.

Splenectomy may be beneficial, but it is generally reserved for refractory cases or to prevent relapses.

Antiplatelet agents (e.g., dipyridamole and aspirin) are of unproven benefit.

Platelet transfusion is of no benefit and may actually lead to worsening of symptoms, although the cause of this is unclear.

A 44-year-old Caucasian woman is admitted to the hospital with a severe nosebleed. The patient states that there was no history of trauma and she never has had nosebleeds before. She also reports a history of upper respiratory infection (URI) symptoms 1 week ago. The patient's blood work is notable for a platelet count of 10,000/cmm. A presumptive diagnosis of idiopathic thrombocytopenic purpura (ITP) is made, and the patient is treated with steroids. The patient's platelet count is refractory and a splenectomy is performed. After a few months, what would you expect to see in this patient's peripheral blood smears?

Answer Choices
1 Burr cells
2 Howell-Jolly bodies
3 Hypersegmented neutrophils
4 Schistocytes
5 Spherocytes

howell-jolly bodies
Explanation
Howell-Jolly bodies, which is the correct response,are basophilic red cell inclusions commonly found in splenectomy patients. This patient has had a splenectomy after being diagnosed with ITP, and therefore, the most likely finding in this patient's peripheral smear would be Howell-Jolly bodies. Howell-Jolly bodies are round, basophilic staining nuclear fragments of DNA in the red blood cell. This finding is classic in patients who have had splenectomy, and in the case of sickle cell disease in which the spleen auto-infarcts.

Burr cells are triangulated helmet shaped cells that are typically seen in diseases of small blood vessels. They can be seen in a number of different diseases including uremia and pyruvate kinase deficiency

Hypersegmented neutrophils are found in folate and B12 deficiency.

Schistocytes are commonly found in Thrombotic Thrombocytopenic Purpura and Disseminated Intravascular Coagulation. A schistocyte is a red blood cell undergoing fragmentation, or a fragmented part of a red blood cell.

Spherocytes are found in hereditary spherocytosis. These patients have the classic triad of jaundice, gallstones, and anemia.

A 64-year-old man presents to his PCP for the first time in 4 years with a recent history of fatigue with pallor and worsening back pain in the lumbar region for the past 3-4 months. He denies any injury to his back or similar pain in the past. He had a colonoscopy 3 months prior, and 2 benign polyps were removed without other abnormalities. At that time, he also had a PSA level of 3.1 ng/ml. On examination, BP: 143/92 mmHg, P: 75 bpm and regular, Resp: 16/minute, Temp: 97.6 degrees F. Skin exam reveals marked pallor without lesions or bruising. Musculoskeletal exam reveals bony tenderness along lumbar vertebrae without paraspinal muscle tenderness to palpation. Decreased range of motion of spine noted on examination due to pain elicited during examination. The remainder of the examination including digital rectal exam, focal neurological, pulmonary, and cardiac exams are within normal limits.

Question
Which of the following tests will be most helpful in making your diagnosis?
Answer Choices
1 Serum protein electrophoresis
2 Complete blood count with differential and platelets
3 Serum calcium level adjusted for serum protein
4 Total serum protein
5 Urine albumin

serum protein electrophoresis
Explanation The correct answer is serum protein electrophoresis as your index of suspicion is high for Multiple Myeloma (MM) based on the case history and physical exam. Prostate and colon cancers are unlikely as the rectal exam and colonoscopy did not yield any significant findings. Typical presenting signs and symptoms include fatigue, pallor, back pain, and occasionally infection, and the median range for this disease is 65 years. Anemia is almost always present and serum protein electrophoresis will almost always include a paraprotein usually IgG, occasionally IgA and light chains. Bone marrow biopsies are also performed to aid in the diagnosis of MM.
A complete blood count is an important first step to look for anemia and other blood cell dyscrasias, but is not specific for MM. Elevated total serum protein and albumin levels are often found in patients with MM but are not specific findings. Serum calcium levels are often found to be elevated at the time of diagnosis but also not specific for MM.

A 46-year-old female patient underwent elective cholecystectomy. The attending nurse noted mild bleeding at the site of IV line and the incision site during dressing. The patient also complained of bleeding from the gums and nose. Coagulation profiles revealed prolongation of aPTT, PT, and TT along with decreased fibrinogen level and increased levels of fibrinogen degradation product (FDP). Platelet count was also decreased. The patient was not suffering from any bleeding disorder previous to her hospitalization.

Question
What is the most likely cause of this patient's bleeding tendency?

Answer Choices
1 Deficiency of clotting factors of the intrinsic pathway
2 Deficiency of clotting factors of the extrinsic pathway
3 Platelet functional defect
4 Disseminated intravascular coagulation (DIC)
5 Cholestatic jaundice

Disseminated intravascular coagulation (DIC)
Explanation
Prolongation of aPTT, PT, and TT along with decreased fibrinogen level, increased levels of FDP, and decreased platelet count indicate disseminated intravascular coagulation (DIC). DIC is an acquired syndrome characterized by bleeding from multiple sites, ecchymoses, mucosal bleeding, and depletion of platelets and clotting factors in the blood. The causes of DIC include infections, bacteria and their toxins, fungi, viruses and rickettsiae, obstetric and gynecological causes such as abruptio placenta, abortions, eclampsia, amniotic fluid embolism, and hemorrhagic shock, malignancy, snake venoms, trauma and transfusions. Diagnostic findings include coagulation abnormalities such as prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin time (TT), decreased fibrinogen levels, increased levels of FDP presence of D-dimer, and decreased platelet count. The mainstay of management involves treatment of the underlying disease. Additionally, blood products such as platelet concentrates, fresh frozen plasma, or cryoprecipitate can also be administered.

The coagulation abnormalities seen in this patient are not associated with deficiency of clotting factors of the intrinsic or extrinsic pathway, platelet functional defect, or cholestatic jaundice.

A 1-year-old boy appears to be physically underdeveloped. His vital signs are not remarkable. His parents are concerned about the boy's health because he seems to be weak and lethargic. The parents are recent immigrants from southern Italy. A brief history check reveals that both parents have a history of mild anemia. The child is afebrile. A complete blood count (CBC) is ordered and the following results were obtained: red blood cell (RBC) count of 3.0x 1012/l; Hb of 8.0 gm/dl; Hct of 24%; peripheral smear showed 1+ basophilic stippling; hypochromia; and the presence of codocytes.

Question
Based on the clinical presentation, medical history of the parents, and the CBC results, what is the most likely diagnosis?

Answer Choices
1 Thalassemia
2 Aplastic anemia
3 Megaloblastic anemia
4 Paroxysmal nocturnal hemoglobinuria
5 Sickle cell anemia

thalassemia
Explanation
Thalassemia is an anemia that is hypochromic and microcytic. Basophilic stippling is often observed. It is an inherited disorder of hemoglobin synthesis characterized by a diminished synthesis of the beta chains, and compensatory synthesis of alpha chains. The ineffective erythropoiesis and hemolysis causes an increased proliferation of the erythroid cells and skeletal changes due to extramedullary hematopoiesis. The peripheral smear shows presence of codocytes and dacrocytes along with the basophilic stippling of the RBCs. Beta-thalassemia is confirmed by hemoglobin electrophoresis. The genotype will determine the severity of the disease. In severe cases, the patients require frequent transfusions, which may eventually lead to iron overload as a complication. Splenectomy is usually performed after the age of 6-7 in order to reduce transfusion requirements.

Sickle cell anemia is characterized by the presence of sickled RBCs in the peripheral blood. The sickling results from the substitution of valine for glutamic acid in the hemoglobin molecule. This is a hemolytic anemia seen mainly in patients of African descent. Stressful conditions, such as hypoxia, may cause sickling and thus sludging of erythrocytes. This sludging leads to infarction of the lungs, spleen, or kidneys in homozygous individuals. The peripheral smear contains sickled RBCs, poikilocytosis, Howell-Jolly bodies, and target cells. The reticulocyte count is usually increased.

Aplastic anemia is characterized by a slight macrocytosis and usually decreased reticulocyte count. There is a reduction in all blood formed elements leading to pancytopenia. Hematopoietic tissue function is affectively reduced. The reduced hematopoietic tissue function is, many times, due to a reaction to chemicals or drugs. However, neoplasia, pregnancy, infectious processes, radiation, and immunological reactions can also produce aplastic anemia.

Megaloblastic anemia is caused by an impairment of DNA synthesis by any of several causes (vitamin B12 deficiency; folate deficiency; and a host of other inborn errors of metabolism). The symptoms include weakness, palpitation, easy fatigability, light-headedness, and shortness of breath. There is severe pallor present; slight jaundice and congestive heart failure can also develop. Glossitis, weight loss, and neurological abnormalities can also be present. B12 or folate supplementation will reverse the anemia, except for the unresponsive form in which the etiological mechanism must be investigated (such as inborn errors of metabolism etc.).

Paroxysmal nocturnal hemoglobinuria (PNH) is a condition characterized by RBC breakdown and release of hemoglobin into the urine, manifested as dark-colored urine in the morning. This is a clinical syndrome characterized by a triad of hemolytic anemia, pancytopenia, and thrombosis. The disorder mainly afflicts young adults, and the chief symptom is irregular episodes of hemoglobinuria that often occur in the morning. There are 2 main causes thought to produce this disorder: an abnormality of the red blood cell membrane and the hemolytic action of complement on the red blood cells. The sucrose hemolysis test and Ham's test can be used to help in the differential diagnosis.

A 40-year-old Caucasian man presents with rapidly enlarging skin lesion on his right shoulder. Upon further questioning, he admits to long-term outdoor exposure as required by his occupation as a construction worker. He further notes that the lesion has been changing colors over the last 2 months, first beginning as solid black then evolving to light brown and red, but he denies any bleeding or pain to the area. Physical exam confirms a 7 mm macular lesion with irregular borders and a mottled color pattern on the right shoulder.
Question
What risk factor serves as the best predictor of metastatic potential of this lesion?

Answer Choices
1 Lesion location
2 Presence of ulceration
3 Color variegation
4 Breslow thickness
5 Serum LDH levels

breslow thickness
Explanation
This patient's presentation is most consistent with malignant melanoma. There are 4 major types of melanoma; 3 of these are characterized by a period of superficial, "radial" growth, during which it increases in size but does not penetrate deeply. These 3 types are superficial spreading melanoma (the most common type of melanoma), lentigo maligna melanoma, and acral lentiginous melanoma. It is during this period that these variants of melanoma are most capable of being cured by surgical excision.

The best predictor of metastatic risk is the lesion's Breslow thickness.

The Clark level, which defines melanomas on the basis of the layer of skin to which a melanoma has invaded, does not add significant prognostic information and is only used in the staging of thin (T1) melanomas.

Other important factors recognized via the staging classification include presence of ulceration, evidence of nodal involvement, serum lactate dehydrogenase (LDH), and presence and site of distant metastases. Another determinant is anatomic site; favorable sites are the forearm and leg (excluding the feet), and unfavorable sites include the scalp, hands, feet, and mucous membranes.

A 41-year-old woman is found to have anemia with Hb of 10g/dl on a routine physical exam and labs. Patient has a history of hypercholesterolemia, depression, and rheumatoid arthritis. She is on a low cholesterol diet, escitalopram (Lexapro) 10 mg daily, and diclofenac 50mg twice daily. Physical examination was essentially unremarkable except for mild arthritic changes at the proximal interphalangeal joints of both hands. Further labs reveal normal MCV and MCHC on peripheral smear; serum iron and TIBC levels are low; percentage saturation with iron is normal; and ferritin level is moderately high. Which is the most likely condition?
Answer Choices
1 Anemia of chronic disease
2 Pernicious anemia
3 Thalassemia
4 Iron deficiency anemia
5 Aplastic anemia

Anemia of chronic disease
Explanation Iron deficiency anemia is the most common form of anemia in USA, where almost 20% women are said to be iron deficient. It is most commonly caused in young to middle aged women due to excessive blood loss during menstruation or as a result of successive pregnancies with poor antenatal care as in the developing countries. Blood loss could also be from a peptic ulcer or GI tumor, e.g. colon cancer. Symptoms occur insidiously with fatigue and mild dyspnea, irritability, and weakness. Nails may become brittle and spoon shaped, also known as koilonychia. Epithelial changes from iron deficiency may cause angular stomatitis, glossitis, and koilonychia; these findings are clinically apparent. The rare clinical triad of cervical dysphagia, iron-deficiency anemia, and the presence of esophageal webs is referred to as the Plummer-Vinson or Patterson-Kelly syndrome. Associated findings include cheilitis, glossitis, and koilonychia. The pathogenesis of Plummer-Vinson syndrome remains enigmatic. Iron therapy can improve esophageal motility in affected patients. Chronically affected patients may require mechanical esophageal dilatation. All of these epithelial alterations are rare in children.
The most devastating consequence of iron deficiency is neurocognitive deficits, which affect children of all ages. Infants and toddlers, in whom such deficits may be irreversible, are vulnerable. Transferrin receptors that are expressed on endothelial cells of the cerebral microvasculature are the gateways for iron entry into the brain. Iron is critical for oligodendrocyte and dopaminergic metabolism. Therefore, iron deficiency can result in hypomyelination, which may be the basis of neurocognitive deficits. Labs typically show microcytosis and hypochromia, as in this patient. Iron studies show low iron and high binding capacity. Low ferritin is the most specific blood test for iron deficiency. High ferritin levels can occur in several different conditions and is non specific. Apart from enriching the diet with iron-rich foods, ferrous sulfate tablets can be given. It is given for 2-3 months (325 mg thrice a day) until significant improvement in Hb is seen.

Anemia of chronic disease is characterized by HB of 10-11g/dl in mild cases; however, 20% patients may have a Hb of <8g/dL. It is associated with any chronic disease like collagen vascular diseases, renal failure, diabetes mellitus with nephropathy, heart diseases, other inflammatory conditions, and malignancies. The anemia is not due to iron deficiency but due to diminished iron utilization by the bone marrow or impaired marrow response to erythropoietin. Labs show normocytosis and normochromia. Serum iron level may be normal or slightly low; TIBC is low; and ferritin is normal or high. Correction of the underlying disease is the treatment.

Pernicious anemia is a megaloblastic anemia due to vitamin B12 (cobalamin) deficiency. There is a lack of intrinsic factor necessary for B12 absorption in the terminal ileum. Folic acid deficiency due to inadequate intake or decreased absorption can also cause megaloblastic anemia as in alcoholics. However, vitamin B12 has a neurological function, and its deficiency is associated with neurological signs and symptoms like peripheral nerve damage, causing paresthesias, posterior column deficits like ataxia, and in severe cases, cerebral deficiencies like dementia and other neuropsychiatric symptoms. The Schilling test is used to diagnose pernicious anemia. After a loading dose of intramuscular vitamin B12, an oral dose of radiolabeled vitamin B12 is given, and excretion in urine is measured. Due to saturated plasma transport proteins from the intramuscular vitamin B12, normally more than 7% is excreted. If there is decreased absorption, then less than 3% is excreted in the urine. In the second step, oral radiolabeled vitamin B12 is given with intrinsic factor, and excretion in urine is measured. If there is a deficiency of intrinsic factor, as in pernicious anemia, then more vitamin B12 will be absorbed and a higher percentage excreted in the urine. A combination of macrocytic anemia, neural deficits, and atrophic glossitis is indicative of pernicious anemia. Labs show large red cells (macrocytes) in the peripheral smear with hypersegmented neutrophils and occasional megaloblasts. Serum iron studies are normal. Antibodies to the intrinsic factor may be found in the blood. Treatment is with parenteral vitamin B12.

Thalassemias are genetic disorders characterized by decreased synthesis of one of the globin chains due to abnormalities in the genes responsible for the synthesis of the globin portion of the hemoglobin molecule. They are named according to the deficient chain. Alpha thalassemias are due to deficient alpha chain synthesis. 4 syndromes can occur, varying from mild asymptomatic anemia to hydrops fetalis. Beta thalassemias are characterized by deficiency of beta chains. Beta thalassemia major is a severe transfusion dependent childhood disease and beta thalassemia minor is a mild anemia agreeable with a full normal life. Labs show microcytosis and hypochromia with bizarre red cell morphology, poikilocytosis, target cells, etc. High red cell turnover may cause high serum iron levels and ferritin. Blood transfusions may result in iron overload.

Aplastic anemia is a disorder of stem cell failure occurring in patients of all ages, with hypocellularity of bone marrow without chromosomal abnormalities. It can be caused by drugs, radiation, and viral infections. CBC shows pancytopenia with reticulocytopenia due to marrow failure. Peripheral blood smear shows normal morphology of cells. Treatment includes removal of offending agents, supportive care, hematopoietic cell transplant, androgens, and chemotherapeutic agents. Iron studies are not helpful because the indices are normal.

A 22-year-old man presents to his physician with swollen lymph nodes in the right axilla. He notes that he develops pain in the area after drinking alcohol. He has been feeling fatigued for the last few weeks and has lost weight without trying. Examination confirms lymphadenopathy in the right axilla. Biopsy of the region shows the presence of Reed Sternberg cells.

Question
What is the treatment of choice at this time?

Answer Choices
1 Lenalidomide and prednisone
2 Doxorubicin, bleomycin, vinblastine, and dacarbazine
3 Radiation therapy
4 Stem cell transplant
5 Observation

Doxorubicin, bleomycin, vinblastine, and dacarbazine
Explanation
The combination of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) is the treatment of choice in Hodgkin's lymphoma. The patient's presentation of lymphadenopathy with the presence of Reed Sternberg cells on biopsy is classic for Hodgkin's lymphoma.

The combination of lenalidomide and prednisone is typically used in the treatment of multiple myeloma, not Hodgkin's lymphoma

Radiation therapy may be used in the treatment of Hodgkin's lymphoma. However, it is usually used in combination with chemotherapy.

Stem cell transplant may be used in the treatment of Hodgkin's lymphoma, but it is not first line.

Observation is incorrect. Hodgkin's lymphoma requires treatment

A 63-year-old woman presents for her yearly exam. She has been feeling well and does not have any current complaints. She has past medical history of hypertension and hyperlipidemia. Routine laboratory testing reveals leukocytosis with 10% blasts. There is mild anemia, and platelets are normal. Peripheral blood cytometry confirms the diagnosis of chronic lympocytic leukemia.

Question
What is the appropriate treatment at this time?

Answer Choices
1 Imatinib
2 Lenalidomide
3 Prednisone
4 Stem cell transplant
5 Observation

observation
Explanation
Observation is correct. Chronic lymphocytic leukemia (CLL) is a malignancy that causes the accumulation of B lymphocytes. It typically follows an indolent course. Treatment is only required for patients who are symptomatic, have extremely elevated WBC counts (greater than 100,000), or have severe anemia or thrombocytopenia. If treatment is required, patients typically receive fludarabine and rituximab, plus or minus cyclophosphamide and prednisone.

Imatinib is used in the treatment of chronic myeloid leukemia (CML), not CLL.

Lenalidomide is usually employed in the treatment of multiple myeloma, not CLL.

Prednisone is sometimes used in the treatment of CLL. This patient is asymptomatic and does not require treatment. If the patient became symptomatic, prednisone would not be used as monotherapy, but would be used in combination with chemotherapy, rituximab, and possibly cyclophosphamide.

Stem cell transplant may be used in the treatment of CLL. However, this is typically reserved for patients who are unresponsive to standard treatment.

Patients with ITP (immune thrombocytopenic purpura) are at risk of spontaneous internal bleeding when the platelet count goes below what level?

Answer Choices
1 50,000
2 30,000
3 10,000
4 2,000

10,000
Explanation
Internal bleeding spontaneously occurs below 10,000.

Bleeding may occur with minor trauma when the platelet count drops below 50,000. Bruising and petechia are seen below 30,000.

A 62-year-old man presents to your office for a follow-up visit. He was diagnosed with acute myelogenous leukemia (AML) 6 months ago and is currently in remission following induction chemotherapy. He is asymptomatic at this time, and his blood counts are normal. However, he wants to know about his outlook for the future.

Question
What is the most important predictor of long-term survival in AML?

Answer Choices
1 The patient's age
2 The patient's sex
3 The subtype of AML
4 The presence of chromosonal abnormalities
5 The duration of remission

the duration of remission
Explanation
Several prognostic factors are important in AML.

The best prognostic factor is the duration of remission after induction therapy. Patients whose remission lasts for 2 or more years achieve second remissions in 50% to 60% of cases, and have a 20% to 25% chance of living at least 3 years after diagnosis. Patients whose remissions last for shorter periods have little chance of surviving for 3 years.

Leukemias in general are more common in males than females, but sex does not appear to play a role in prognosis once diagnosed.

Older patients have a lower median survival than do younger patients. Part of this may be explained by the fact that older patients tend to have chromosomal abnormalities involving deletions, as opposed to the translocations more commonly seen in younger patients. Cases of AML involving chromosomal deletions are generally less responsive to treatment and have a poorer prognosis. Another factor accounting for poorer survival among older patients may be the inability to pursue aggressive therapy. This is usually due to comorbid diseases.

The subtype of AML can also be important. Acute promyelocytic leukemia (APL) carries a better prognosis than other subtypes of AML. Generally, the more mature the cell of origin, the longer the median survival.

A 12-year-old Jewish girl is brought in by her father. The father reports that the child started to bleed profusely following tooth extraction 2 hours ago. Further questioning reveals that there is a history of bleeding disorder in the child's maternal uncle and aunt. Examination reveals tachycardia and bleeding from the site of tooth extraction. Coagulation profile reveals prolonged activated partial thromboplastin time (aPTT), normal prothrombin time (PT) and thrombin time (TT), and deficiency of factor XI. The child was treated with fresh frozen plasma (FFP) and fibrin glue.

Question
What is the likely diagnosis?

Answer Choices
1 Thrombocytopenia
2 Hemophilia B
3 Von Willebrand's disease
4 Hemophilia A
5 Hemophilia C

Hemophilia C
Explanation
Hemophilia C is an inherited autosomal disorder characterized by deficiency of factor XI and is common in Ashkenazi or Iraqi Jews. Patients are usually asymptomatic and spontaneous bleeds into joints, soft tissues, and muscles are rarely seen in Factor XI deficiency. Bleeding usually occurs after tooth extractions, trauma, or surgery, such as tonsillectomy. Hemophilia C should be suspected in patients with prolonged aPTT and a positive family history of life-long bleeding disorder affecting both the male and female family members. This condition is primarily treated with FFP and other adjunctive measures such as fibrin glue and antifibrinolytics. Replacement with Factor XI concentrates can be done when available.

In addition to general supportive therapy, a cryoprecipitate or factor VIII concentrates are recommended for the treatment of hemophilia A, an X-linked disorder that results in deficiency of Factor VIII. The diagnostic characteristic is a decrease in Factor VIII. Patients who bleed frequently even without discernible trauma usually show <1 percent Factor VIII activity. Many patients with mild hemophilia A and most with von Willebrand's disease can be treated with desmopressin (DDAVP).

A deficiency of von Willebrand's factor is characteristic of von Willebrand's disease. In addition to supportive measures and cryoprecipitate, desmopressin (DDAVP) is preferred in most cases. It is often seen in females who started their period and postpartum hemorrhage. Factor VIII and platelet function is affected. It is the most common bleeding disorder.

Christmas disease, hemophilia B, is a Factor IX deficiency that produces a clinical picture similar to hemophilia A. It is diagnosed by the deficiency of Factor IX. The recommended treatment is administration of Factor IX concentrates.

Thrombocytopenia may be congenital or acquired and is characterized by decreased circulating platelets in the blood. The patient presentation, lab tests, and treatment are not suggestive of Hemophilia A, B, thrombocytopenia, or von Willebrand's disease.

A 65-year-old woman presents with pain in the back, chest, and at the right seventh rib. She appears to be quite pale, and she admits to fatigue. These symptoms have come on gradually over a period of several weeks, with the back pain becoming the reason for consulting the physician.

Physical examination reveals localized tenderness at the spine of T8 and ribcage with tenderness at the right seventh rib.

CBC shows a normochromic, normocytic anemia with hemoglobin of 8 g/dL. Peripheral smear shows marked rouleaux formation with normal platelet and white cell counts and morphology. Serum chemistry results include calcium elevation to 12.2 mg/dL with normal alkaline phosphatase. A dipstick urinalysis shows proteinuria.

A bone marrow aspirate was dry. The bone marrow biopsy is pending.

Plain film X-rays of the chest show a fracture of the right seventh rib and compression of the eighth vertebra.

Question
What is the most likely diagnosis?

Answer Choices
1 Chronic lymphocytic leukemia (CLL)
2 Chronic Myelogenous Leukemia (CML)
3 Hairy-cell leukemia (HCL)
4 Multiple myeloma
5 Myelofibrosis

Hairy cell-leukemia
Explanation
Hairy-cell leukemia (HCL) is a rare B cell neoplasm that most often occurs in older men. Patients usually present with symptoms related to impairment of the bone marrow, such as infections and bleeding, although they may be asymptomatic. The physical examination is usually only significant for splenomegaly; laboratory studies typically reveal pancytopenia. The bone marrow aspirate is often dry secondary to fibrosis; biopsy may be needed to make the diagnosis.

Treatment of HCL is indicated only if there is worsening splenomegaly or lymphadenopathy or if there are more than 20,000 hairy cells/μl. The current treatment of choice is either cladribine or pentostatin; both of these agents have been shown to induce remission in 80% of patients. Although splenectomy may improve symptoms, it does not affect the disease itself.

'Chronic lymphocytic leukemia (CLL)' is an incorrect response. Patients with CLL have a higher-than-normal white blood cell count

'Chronic Myelogenous Leukemia (CML)' is an incorrect response. Patients with CML present with splenomegaly, but they have a higher-than-normal white blood cell count.

Multiple myeloma is very unlikely, given the dry marrow aspirate. Patients with multiple myeloma would be expected to a have high plasma cell count on bone marrow biopsy or aspirate.

Myelofibrosis is unlikely. Most patients with Myelofibrosis present with moderate-to-severe anemia, but in contrast, the peripheral smear appears abnormal and is characterized by teardrop shaped red blood cells.

A 3-year-old boy in your practice has sickle cell anemia and is also followed by a pediatric hematologist. He has been admitted to the hospital with a history of three days of fever ranging from 38 to 38.5 degrees Centigrade, a cough, and increased work of breathing. Despite aggressive therapy, the patient continues to have a decline in oxygen saturation level. A decision is made to administer hydroxyurea and to perform an exchange transfusion. What effect would hydroxyurea be expected to have?

Answer Choices
1 Decrease cell adherence
2 Improve bacterial phagocytosis
3 Increase fetal hemoglobin
4 Promote vasodilation
5 Reverse dehydration of sickled red blood cells

Increase fetal hemoglobin
Explanation
Hydroxyurea, first used as a cancer chemotherapy drug, is thought to increase fetal hemoglobin by causing red blood cell regeneration in response to its cytotoxic effects. It is considered a reasonable consideration in painful vaso-occlusive crisis, acute chest syndrome and in stroke. Hydroxyurea may also increase gamma globin gene expression.

Over a nine year period in adults, when hydroxyurea was used at subtoxic doses it decreased the pain from acute chest syndrome by nearly half and decreased the cumulative mortality by 40%. In children, Hb F increased over 20% and no significant toxicity was noted over seven years. Hydroxyurea increases both the percentage of Hb F and the number of cells containing Hb F and also decreases the number of neutrophils, reticulocytes, and platelets. These side effects may cause increased susceptibility to infection and bleeding. Hydroxyurea may also conserve energy and decrease the hypermetabolic state of children with sickle cell disease. Hydroxyurea is potentially mutagenic and carcinogenic.

A 58-year-old male presents to the office for routine follow-up of hypertension.
A CBC on the front of the chart reveals:

WBC 7.0 x 103/mm3 4.5 - 11
RBC 6.5 x 106/μl 4.6 - 6.2 (male)
Hematocrit 58 % 40 - 54 (male)
MCV 68 μ3 80 - 100
RDW 14.9 % 11.6 - 14.6
Platelets 250,000 mm3 150,000 - 450,000
Upon questioning, he admits that he has not been compliant with diet or exercise and continues to smoke. On examination height is 5'8, weight is 210 lbs, with a BMI of 32. Blood pressure is 156/88 mm Hg, pulse is 88/min, temperature 98.6°F, and respirations 16/minute. Skin is dry without rubor or cyanosis. On auscultation, lungs are clear and the heart has a regular rhythm without murmur, gallop, or rub. Abdominal exam reveals truncal obesity, active bowel sounds, and no organomegaly. The remainder of the PE is normal. Office pulse oximetry reveals a saturation of 92%. You send a blood specimen for an erythropoietin level.

Question
In the event the erythropoietin level is normal, the most likely diagnosis is
Answer Choices
1 Polycythemia vera
2 Hydronephrosis
3 Sleep apnea
4 Gaisböck's syndrome
5 Carboxyhemoglobinemia

Explanation The correct answer is Gaisböck's syndrome. This syndrome is prevalent in hypertensive, obese males who smoke and present with erythrocytosis on CBC. This is a relative polycythemia in which there is a normal RBC mass in the presence of decreased plasma volume resulting in a hemoconcentration. Since this is a relative or spurious polycythemia, erythropoietin levels are normal. Other causes of hemoconcentration include burns, stress, diuretics, and decreased oral intake.
Erythropoietin levels are low-to-normal in Polycythemia Vera (PV), but to make the clinical diagnosis of PV, there must be 3 major criteria: increased RBC mass, normal O2 saturation, and splenomegaly. Since splenomegaly was not present, the diagnosis could be made with the first 2 major criteria plus the addition of 2 minor criteria: leukocytosis in the absence of infection and elevated platelets with abnormal function resulting in bleeding or thrombosis. In this patient, the leukocyte and platelet counts are normal, so the criteria for diagnosing PV are not met. Likewise, he lacks the characteristic rubor of extremely high levels of erythrocytosis.

Kidney diseases, such as hydronephrosis or renal cysts, and erythropoietin-secreting renal tumors can produce an inappropriate rise in erythropoietin levels and a subsequent, secondary erythrocytosis.

Carboxyhemoglobinemia could be present as a result of smoking, but would cause tissue hypoxia, as would sleep apnea. Tissue hypoxia increases production of erythropoietin resulting in a secondary, compensatory erythrocytosis. This patient had a normal pulse oximetry saturation and no evidence of cyanosis.

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