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PANCE Topic List: Heme/Onc

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Characteristics of an RBC
98% hemoglobin
Biconcave disc
Made in bone marrow.
Lasts 120 days, destroyed in the spleen
What are the best initial diagnostic tests for RBC disorders? Other tests?
CBC and peripheral smear
Other: Retic count, UA, BMP
What is hemoglobin? What is hematocrit?
Hemoglobin is a tetramer protein made of 2 alpha and 2 beta chains that transports O2. Hematocrit is the % of RBCs in blood.
Normal H&H in males and females?
Males: Hct 41-53%, Hgb 13-17
Females: Hct 36-46%, Hgb 12-16
What is RDW?
The red cell distribution width (RDW) is a measure of the variability in size of red blood cells. It is high in iron deficiency anemia
What is mean corpuscular volume (MCV)? What is it helpful for?
Average size of RBC - helpful tool in diagnosis of low production anemias. Low MCV (<80) = microcytic anemia. High MCV (>100) = macrocytic anemia
What is the Direct Coombs test?
Done to detect cell lysis resulting from an autoimmune process. It detects the presence of antibodies directed at a patient's own red blood cells.
What is Hemoglobin electrophoresis?
Used in the diagnosis of thalassemias and sickle cell disease to detect different types of hemoglobin
What is Anemia?
A condition where there is a decrease in the number of RBCs, hemoglobin, or hematocrit due to either increased destruction/loss or decreased production of RBCs
How are Anemias classified?
By their pathophysiologic basis: either diminished production of RBCs or accelerated loss of RBCs
How do you differentiate between diminished production of RBCs and accelerated loss of RBCs?
Retic count. A reticulocyte is a baby RBC. If retic count is high, production of RBCs are high which means there is an accelerated loss of RBCs through either bleeding or hemolysis.
What hormone stimulates production of RBCs? Where is it made?
Erythropoietin (EPO); kidneys - therefore renal failure can cause anemia
What are clinical features of anemias?
Weakness/fatigue
Palpitations
Angina
Dyspnea on exertion
Positional dizziness
Tinnitus
Syncope
Bleeding
PE findings with anemia?
Pallor
Koilonychia (spoon nails)
Palmar creases
Increased cardiac output/"flow" murmur
Tachycardia
Orthostatic hypotension
Petechiae or purpura
Jaundice
Glossitis or angular chelitis
Thyroid enlargement
GI bleed
Increased vibratory or position sense
What are 4 microcytic anemias? Which is the most common and leading cause of anemia worldwide?
TICS:
Thalassemias
Iron deficiency <---most common
Chronic inflammatory block (Anemia of chronic disease)
Sideroblastic anemia
What is the most frequent cause of iron deficiency?
Blood loss
What is a pathognomonic symptom of iron deficiency anemia?
Pica - Craving/chewing on things with no nutritional value - Ex: ice, clay
What are common lab/peripheral smear findings with iron deficiency anemia?
Low MCV
Low Iron
Low Ferritin
Low % sat
Low Retic count
High TIBC
High Transferrin
Peripheral smear: Hypochromic RBCs, Tear drop cells, elliptocytes, anisocytosis [red cells are unequal size (high RDW)]
What is Ferritin indicate? What is TIBC?
Ferritin represents iron stores. Ferritin will be low with iron def. anemia because iron stores are low

TIBC is total iron binding capacity - a protein carries iron around in the blood. TIBC will be high with iron def. anemia because there is a low amount of iron to bind to TIBC
Ferritin of <____ is pathognomonic of iron deficiency?
15
What is the definitive test for iron deficiency anemia?
Bone marrow biopsy
How do you treat iron deficiency anemia? What increases iron aborption?
Oral Iron (Ferrous sulfate 325 PO TID) or Parenteral Iron. Ascorbic acid (Vit C) doubles absorption.
AE of Iron?
GI side effects: metallic taste, N/V/D, flatulence, constipation, epigastric distress
Black/green or tarry stools
How long does it take to correct iron def. anemia with iron supplementation? How long should it be continued?
6 weeks. 6 months after renewal of stores. Test of choice to measure progress is retic count.
Clinical features of iron overdose?
Iron causes localized necrosis and hemorrhage at the point of contact in the GI system resulting in abdominal pain, vomiting, bloody diarrhea, and hematemesis.
What is thalassemia?
Group of hereditary disorders characterized by decreased of absent synthesis of alpha or beta globin chains
How are thalassemias categorized?
Type: A (alpha chain is affected) or B (beta chain is affected)
Severity
What are the three levels of severity with thalassemia?
Trait - no clinical impact
Intermedia - there are RBC tranfusion requirements
Major - Life threatening
Clinical features of alpha thalassemia?
A mutation that deletes one or more of the a-chain genes on chromosome 16
Clinically milder than beta thalassemia
More common in Asians
Clinical features of beta thalassemia?
Caused by a point mutation
2 types
More common in Mediterranean and African populations
More severe disease and presents early in life (6 months)
What are the 2 types of beta thalassemia and clinical features of each?
Minor: Increased level of HgA2, Target cells.

Major (AKA Cooley's anemia): More severe. Increased level of HgbF.
Labs/peripheral smear findings for thalassemias?
Low MCV
Normal Iron
Normal TIBC
Normal % sat
Normal ferritin
Abnormal Hgb electrophoresis
Elevated LDH
Peripheral smear: Elliptocytes, Target cells
Best test for diagnosing thalassemias?
Hemoglobin electrophoresis
How do you treat thalassemias?
If mild: Genetic counseling and education (avoid iron)

If severe:
Folate
Transfusions
Iron chelation therapy
Bone marrow/stem cell transplant
Splenectomy
What are common causes of anemia of chronic disease (ACD)?
Secondary to...
Chronic infection
Inflammation
Cancer
Liver disease
ESRD
Labs/peripheral smear findings for ACD?
Can be microcytic or normocytic
Low Iron
High Ferritin
Low Transferrin
How do you treat ACD?
Treat underlying cause
Give EPO
What is Sideroblastic anemia?
Anemia due to mitochondrial defect which prevents the incorporation of iron in to hemoglobin. Iron accumulates in the mitochondria and forms a "ringed sideroblast"
Causes of sideroblastic anemia?
Acquired
Hereditary
What are causes of acquired sideroblastic anemia? What is MC?
Excessive alcohol use <---MC
B6 (pyridoxine) deficiency
Lead poisoning
Copper deficiency
Lab/peripheral smear findings with sideroblastic anemia?
Low MCV in hereditary
High MCV in acquired
High Ferritin
High Iron
Low TIBC
Ringed sideroblasts are seen
Treatment of sideroblastic anemia?
B6 supplementation
What are the macrocytic anemias (MCV>100)?
Megaloblastic (Folic acid anemia, Vitamin B12 anemia)
Non-megaloblastic
Clinical features of lead poisoning?
Abdominal pain
Constipation
Peripheral neuropathy
Neuro changes
Treatment of lead toxicity?
Remove patient from environment
Chelation therapy if severe
What is the pathogenesis of megaloblastic anemia?
There is impaired DNA synthesis because the cytoplasm matures faster than the nucleus which leads to ineffective RBC formation and macrocytosis.
How is B12 absorbed?
Binds to intrinsic factor which goes to the intestine and is absorbed in the ileum and is stored by the liver
Where is B12 found?
Only found in foods of animal origin
What are causes of B12 deficiency? (9)
Pernicious anemia
Malnutrition (alcoholics, anorexic, elderly)
Strict diet (Vegetarian, Vegan)
Bariatric surgery
Hereditary
Severe Crohn's disease
Celiac disease
Tapeworm
Pancreatic insufficiency
What is the most common cause of B12 deficiency?
Pernicious anemia - lack of intrinsic factor due to a hereditary autoimmune disease that usually doesn't present until after age 35
What are hallmark clinical features of B12 deficiency?
Beefy, red tongue
Glossitis
Neurologic symptoms (stocking glove paresthesias, loss of position, fine touch, and vibratory sense, ataxia)
What is the pathognomonic finding of lead toxicity?
Basophilic stippling
What are common lab findings with B12 deficiency?
High MCV
Low B12
Anisocytosis and poikilocytosis
Pancytopenia
Increased LDH
Mildly increased Bilirubin
HIGH Homocysteine and Methylmalonic acid (MMA) levels
Hypersegmented Neutrophils
How do you treat B12 deficiency?
1mg B12 IM injections - if due to malnutrition
Oral therapy - if due to decreased intake (vegetarians)
What could happen if B12 deficiency goes on for too long?
Irreversible dementia and neurologic symptoms
(note neuro deficits are reversible if treated within 6 months)
What does folic acid (B9) do in the body and where is it found?
Cellular reactions, DNA synthesis
Found in fruits, vegetables, cereals, bread
What is the most common cause of folate deficiency?
Inadequate dietary intake or to increased use, such as in pregnancy
Hallmark findings with folic acid deficiency?
Glossitis
NO NEURO SX
Vague GI symptoms
What are lab findings with folic acid deficiency?
High MCV
HOWELL-JOLLY bodies (nuclear DNA remnants)
Normal B12
Low Folic Acid
High Homocysteine
LOW-normal MMA!
Oval macrocytic RBCs
Hypersegmented Neutrophils
Tx for folic acid deficiency?
1mg/day of folic acid
Avoid alcohol and certain meds
What is important to r/o when treated folic acid deficiency?
B12 deficiency
Normocytic normochromic anemias
ACD
Hemolytic anemias
Anemia of hemorrhage
Aplastic anemia
What is Aplastic anemia?
A decrease in production of all cell lines due to damage to stem cells
Causes of aplastic anemia?
Acquired
Hereditary
What is the MC form of hereditary aplastic anemia?
Fanconi anemia. Affects children Characterized by pancytopenia with macrocytic anemia and congenital malformations (short stature, hypopigmented spots and café-au-lait spots, abnormality of thumbs, microcephaly or hydrocephaly, hypogonadism, and developmental delay)
What are the characteristic lab findings in aplastic anemia?
Pancytopenia and a hypocellular bone marrow
Treatment of aplastic anemia?
Bone marrow transplant
What are the hemolytic anemias?
Sickle Cell disease (SCD)
G6PD deficiency
Hereditary Spherocytosis
Polycythemia vera
What is hemolysis?
Premature destruction of RBCs in the reticuloendothelial system or in the blood vessels. Bilirubin will be elevated from break down of RBCs
What does chronic hemolytic anemia lead to?
Jaundice due to increase in indirect bilirubin
Pigment gallstones
Splenomegaly
Poorly healing ulcers over tibia
Petechiae and purpura
What are common lab findings for all hemolytic anemias?
Haptoglobin is depressed
High indirect and total bilirubin
Elevated LDH
Elevated retic count
Falling Hgb
Peripheral smear will show SHISTOCYTES
What serum blood test helps determine the degree of hemolysis?
Haptoglobin - decreased with inherited hemolytic anemias
Which type of Coombs test would you do to evaluate hemolytic anemia?
Direct - Mix the patient's RBC with a reagent and look for IgG and C3
What is Polycythemia vera?
Disorder of the bone marrow which causes too many RBCs (HCT > 60%) to be produced. The numbers of WBCs and platelets may also be higher than normal. Rare. M>F
What causes polycythemia vera?
JAK2 mutation
Clinical features of polycythemia vera?
HA
Dizziness
Tinnitus, blurred vision, fatigue
Epistaxis (due to mucosal engorgement and platelet dysfunction)
Erythromelalgia (burning pain in feet or hands with erythema, pallor, and cyanosis)
PRURITIS following warm showers
PE findings with polycythemia vera?
Plethora
Engorged retinal veins
Splenomegaly
Hypertension
Treatment for polycythemia vera?
Serial phlebotomy
Hydroxyurea, aspirin, allopurinol
Complications from polycythemia vera?
Thrombosis <---MC
Transformation to acute myelogenous leukemia (AML)
What is Glucose-6-phosphate dehydrogenase deficiency (G6PD deficiency)?
Hereditary disorder which is X linked and most commonly seen in AA males. It is the MC disease-producing enzymopathy in humans. There are more than 300 variants of the disease and some protection against malaria which likely contributes to its high frequency in the population. Oxidative stress causes hemolysis.
What does G6PD do?
Catalyzes two important reactions: the oxidation of G6P and also the reduction of NADP+ to NADPH. NADPH is a required co-factor in many enzymatic reactions in the body. In red cells, NADPH protects the cells against oxidative stress and therefore is heavily reliant on G6PD. In the presence of oxidative stress and oxidative pharmacologic agents, red blood cells begin to rapidly hemolyze.
What are causes of oxidative stress that lead to hemolysis in G6PD deficiency?
Meds (Sulfonamides, Antimalarials, Dapsone, Aspirin, Nitrofuratoin)
Infection
DKA
FAVA BEANS!
How do patients present with acute hemolytic crisis?
Jaundice
Anemia
Splenomegaly
Vascular collapse
What lab findings will you see with G6PD?
HEINZ bodies - denatured Hgb
BITE CELLS - look like a bite has been taken out of them
High retic count
High indirect bilirubin
Low G6PD levels
Low Hematocrit
Decreased haptoglobin
How do you treat G6PD?
Self-limiting. Pts are usually asymptomatic in between attacks.
Avoid oxidative drugs and food
What is hereditary spherocytosis?
Hereditary disorder of the RBC membrane scaffolding proteins (actin or spectin) which leads to chronic hemolytic anemia.
What does the defect do to the morphology of the RBC?
Causes it to become spherical (spherocyte) which is less deformable and unable to squeeze through capillaries and they hemolyze
How do patients with hereditary spherocytosis present?
+ family history
May or may not have anemia
Splenomegaly
Chronic hemolysis which causes jaundice and gallstones
What test confirms the presence of spherocytes?
Osmotic fragility test - involves incubating red blood cells in hypotonic saline in increasing dilutions. This causes the red blood cells to swell and eventually burst. The spherocytes more readily lyse than normal cells in hypotonic solutions. Not a specific test however.
What lab findings will you see with hereditary spherocytsosis?
SPHEROCYTES
High Retic count
Increased MCHC
Microcytic, hyperchromic cells
Increased indirect bilirubin
Negative Coombs test
Children with hereditary spherocytosis are at risk for aplastic crisis as result of an infection from which virus?
Parovirus B19
How do you treat hereditary splenocytosis?
TOC: Splenectomy
Folic acid supplementation
What is sickle cell disease (SCD)?
Autosomal recessive disorder where abnormal hemoglobin leads to chronic hemolytic anemia and microvascular occlusion
What are the hallmarks of SCD? (4)
Recurrent painful episodes
Positive family history
Irreversibly sickled cells
Hemoglobin S seen on electrophoresis
What causes increased sickling?
Concentration of Hgb S and presence of other Hgb in cell
Hypoxemia
Acidosis
Dehydration
Where is the pain of SCD normally felt?
Long bones
Back
Chest
Repeated vascular occlusions damages which organs?
Heart - enlarges
Lungs
Liver - enlarges
Bones
Spleen - eventually infarcts
What lab findings will you see with SCD?
Hct is 20-30%
Sickled cells on smear
Howell-Jolly bodies
WBC elevated
May have thrombocytosis
Elevated indirect bilirubin
What is the diagnostic study of choice for SCD?
Hemoglobin electrophoresis.
How do you treat SCD?
Prevention of infection - vaccinate!
Prevention of vaso-occlusive crises - HYDROXYUREA
MOA of Hydroxurea?
Increases fetal hemoglobin (HbF) production and slightly raises the total hemoglobin concentration in the body. Fetal hemoglobin reduces the chance that RBCs will sickle.
How do you treat SCD crisis?
Treat underlying cause
Hydrate
Oxygen
Transfusion
Analgesia
Sickling complications?
Aplastic crisis: ↓ Hb + reticulocytopenia, parvovirus B19
Acute chest syndrome: Fever, CP, CXR: pulmonary infiltrate (MC cause of death in adults)
Splenic sequestration crisis: rapid splenic sequestration of RBCs → splenomegaly + severe anemia
Stroke
Aseptic necrosis of the femoral head
Dysfunctional spleen → ↑ infection risk
Salmonella osteomyelitis
S. pneumoniae sepsis: MC cause of death in children
What treatments can help prevent stroke in SCD patients?
PCN prophylaxis
PNA vaccine
Transcranial doppler screening
How do clotting and platelet disorders generally present?
With excessive or repetitive bleeding, bruising, or bleeding at unusual sites
Skin petechiae
Increased mucosal bleeding
Hemarthroses and deep tissue hematomas
Purpura from vasculitis
What are the two main categories of disorders of hemostasis (clotting disorders)?
Platelet number or function
Coagulation factor abnormalities
DDx for clotting disorders/thrombocytopenia
PVC Pipes:
Platelets - either not enough or not functioning
vWD
Clotting factor disorders (hemophilias)
Pipes - vasculitis, scurvy, etc
Diagnostic studies for clotting disorders/thrombocytopenia?
CBC
Peripheral smear
Platelet function analysis (PFA)
PT/INR - tests extrinsic clotting pathway
aPTT - tests intrinsic clotting pathway
CMP
D-dimer
What are the coagulation abnormalities?
Von Willebrand disease
Hemophilia A
Hemophilia B
Coagulopathy of liver disease
Vitamin K deficiency
Factor V Leiden
Cancer as a cause of coagulopathy
DIC
VTE/DVT/PE
What is the most common inherited bleeding disorder?
von Willebrand Disease
What is von Willebrand factor (vWF)? What does it do?
A large glycoprotein that aggregates platelets and prolongs the half life of factor VIII
What are the two major types of VWD?
Type 1: Patients have a quantitative abnormality of vWF
Type 2: Patients have a qualitative abnormality of vWF
How does Type 1 VWD present?
Mild to moderate platelet type bleeding (MUCOSAL membranes - epistaxis, gingival bleeding)
What are lab findings of Type 1 VWD?
VWF activity and Ag are mildly depressed
How does Type 2 VWD present?
Moderate to severe bleeding that presents in childhood
What are lab findings of Type 2 VWD?
Ag:vWF activity is 2:1
So they have lots of factor but it doesn't work
How do you treat VWD?
DDAVP for Type 1
Factor VIII replacement if more severe
What is Hemophilia A?
Deficiency of Factor VIII
X-linked recessive disorder - which means only males will get disease and females will be carriers
Most severe bleeding disorder. 2nd most common after VWD.
How do patients with Hemophilia clinically present?
If mild: spontaneous bleeding may occur with trauma or surgery

If severe: spontaneous bleeding into joints and soft tissues (hemarthrosis), epistaxis, intracranial bleeding, melena, gingival bleeding
Where is the MC site of spontaneous hemarthrosis in adults? Children?
Adults: Knee
Children: Ankle
What lab findings will you see with hemophilia A?
Low factor VIII activity
Prolonged aPTT that corrects when mixed with normal plasma
Tx for Hemophilia A?
Factor VIII replacement
DDAVP
Avoidance of ASA
What is Hemophilia B?
AKA Christmas disease
Deficiency of Factor IX
X-linked recessive disorder
Less common than Hemophilia A
Tx for Hemophilia B?
Replace Factor IX
What is the most common acquired coagulopathy?
Vitamin K deficiency - due to poor diet, liver failure, malabsorption, certain drugs
How do you treat Vit K deficiency?
Oral or parenteral Vit K
FFP
Green leafy veggies
What is thrombocytopenia?
<150,000
Severe is <50K
How do you determine the cause of thrombocytopenia?
Bone marrow biopsy
What are 6 major acute thrombocytopenias?
ITP
TTP
HIT
HUS
HELLP
DIC
What is Immune Thrombocytopenic Purpura (ITP)
Self limited autoimmune (IgG) condition where pathogenic antibodies bind to platelets and result in accelerated platelet clearance. Idiopathic cause and also associated with connective tissue disorders, lymphoma, meds, infections, viruses (in children)
Clinical features of ITP?
Sudden appearance of a petechial rash, bruising, or bleeding in an otherwise healthy child with a history of recent viral infection
Cutaneous bleeding (petechiae, purpura, or bruising) is the MC finding (seen in 85% of cases)
Diagnosis of ITP?
The diagnosis of ITP is based upon the following criteria: Platelet count < 100,000/microL with otherwise normal CBC
Normal peripheral blood smear
Tx of ITP?
Usually resolves on its own within 3 mo
Sometimes requires steroids, IVIG, or splenectomy
What is Thrombotic thrombocytopenia Purpura (TTP)
Thrombocytopenia due to platelets making small clots in the microvasculature and microangiopathic hemolytic anemia (RBCs are exploding). Can be congenital or acquired. Due to inhibition of the enzyme ADAMTS13, which leads to large multimers of von Willebrand factor
What is the classic TTP pentad?
Microangiopathic hemolytic anemia
Thrombocytopenia
Fever
Renal insufficiency
Neurologic abnormalities
Cause of TTP?
Idiopathic
Drugs
Autoimmune
Pregnancy
HIV
How is HUS different from TTP?
HUS doesn't have neuro abnormalities or fever.
HUS occurs in children after an E.coli infection
TTP occurs in more commonly in adults
What will be seen on peripheral smear with TTP
Schistocytes (fragmented erythrocytes, also called helmet cells)
How do you treat TTP?
Goal: Remove antibody and replenish cleaving protease activity
Best treatment: Plasmaphoresis (remove plasma and replace with fresh frozen plasma) for 1-8 weeks
Steroids and anti-platelets
What do you not want to do when treating TTP?
Give platelets - causes more clotting
Hypercoagulable states
Factor V Leiden
Prothrombin mutation
Hyperhomocysteinemia
Protein C def
Protein S def
Antithrombin III def
When should you suspect an inherited hypercoagulable state?
In patients with venous or arterial thrombosis at a young age or unusual locations, recurrent thromboses or pregnancy losses, or +FHx
What is leukopenia?
Low number of circulating WBCs (<4.5K) in the bloodstream. Caused by infections, toxins, meds, chemo, leukemias, myelomas, or primary marrow failure.
What are clinical features of leukopenia?
Fever/chills
Opportunistic infections
Signs of malignancy
What is leukemia?
Increased production of abnormal leukocytes and leukocyte precursors in circulation and bone marrow. Four types: AML, ALL, CML, CLL)
How are leukemias classified?
Cell type (myelocytic - ML or lymphocytic LL)
Acute vs chronic
What is the most common leukemia?
CLL
What is the most common leukemia in children?
ALL
What is acute leukemia?
Immature, abnormal cells are in the bone marrow (>20%) and peripheral blood (>10%) and frequently in the liver, spleen, lymph nodes, and other organs. Symptoms occur over days.
What are clinical features of acute leukemias?
Symptoms are related to replacement of normal bone marrow with abnormal WBCs, all cell lines will be affected:
Gingival bleeding
Epistaxis
Menorrhagia
Infections
Fatigue, fever, lethargy, headache, bone/joint pain
Diagnostic studies and findings for acute leukemias?
CBC - will show pancytopenia with blasts
Uric acid - Hyperuricemia
Cytogenic studies
What is acute lymphocytic leukemia (ALL)?
Leukemia that primarily affects CHILDREN (80% are 3-7). 75% affects B-cell precursors
20% T-cell precursors
MOST CURABLE type of leukemia
What are symptoms specific for ALL?
Lymphadenopathy and hepatosplenomegaly
Diagnostic hallmark for ALL?
Terminal deoxynucleotidyl transferase
How do you treat ALL?
Aggressive chemo for 2 years
Consider bone marrow transplant
What indicates a poorer prognostic factor for ALL?
Presence of Philadelphia chromosome
Tx for hypercoagulable states?
Usually asymptomatic
Consider prophylactic anticoagulation if risk factors develop
What is acute myelogenous leukemia (AML)?
Acute leukemia that primarily affects adults (mean age is 60). There is excessive proliferation myeloid precursors cells.
What are myeloblasts?
Immature hematopoietic cells with large nuclei and prominent nucleoli that can develop into basophils, eosinophils and neutrophils.
Diagnostic hallmark for AML?
Auer Rods - pinkish-red rod-like structures in the cytoplasm of myeloblasts
What jagged, irregularly shaped, red blood cells indicate disseminated intravascular coagulation, a complication of acute myelogenous leukemia?
Shistocytes
How do you treat AML?
Aggressive chemo - survival rate is 30%
What are two chronic leukemias?
Chronic lymphocytic leukemia (CLL)
Chronic myelocytic leukemia (CML)
What is CLL?
A clonal malignancy of B lymphocytes. Most common leukemia! More common in men than women. Median age of onset is 65.
What are clinical features of CLL?
Indolent course. Mean survival is 6 years after diagnosis.
Recurrent infections
Splenomegaly
Lymphadenopathy
What is the diagnostic hallmark of CLL?
ISOLATED LYMPHOCYTOSIS (>20K WBCs)
Peripheral smear will show SMUDGE CELLS, increased mature small lymphocytes
Bone marrow is hypercellular with a left shift
Tx for CLL?
Palliative
What is CML?
A myeloproliferative disorder. Median age of onset is 55. Prognosis is better than CLL (80% are alive after 6 years)
What are clinical features of CML?
3 phases (chronic, accelerated, acute):
B symptoms: fatigue, anorexia, weight loss, fever, excessive sweating
Abdominal fullness from splenomegaly
Symptoms develop gradually
Diagnostic hallmark for CML?
PHILADELPHIA CHROMOSOME or BCR-ABL gene
LEUKOCYTOSIS (WBC count >150)
Peripheral smear will show anemia and thrombocytosis
What is standard pharm tx for CML? Curative tx?
Bone marrow transplant <---curative
Chemo - Imatinib (Gleevec) is standard therapy
What is Lymphoma?
A group of blood cell tumors that develop from lymphatic cells.
What are two major lymphomas?
Hodgkin disease (HD)
Non-Hodgkin lymphoma (NHL)
What is Hodgkin disease?
Group of cancers characterized by enlargement of lymphoid tissue, spleen, liver, and presence of REED-STERNBERG cells (bi-lobed nuclei).
Associated with EBV
MC between 15-45 yo
What is the MC presentation of Hodgkin disease?
Painless mass in the neck
What are clinical features of Hodgkin disease?
Painless cervical, supraclavicular, and mediastinal lymphadenopathy
May have painful lymph nodes after ingestion of alcohol
Constitutional B symptoms: Fever, night sweats, weight loss
SOB
Diagnostic studies for Hodgkin disease?
Lymph node biopsy - will show REED STERNBERG CELLS
PET scan
CT of neck, chest, abdomen, pelvis for staging
Tx for Hodgkin disease?
Chemo cures most - Adriamycin, bleomycin, vinblastein, dacarbazine (ABVD)
Radiation <--- TOC for low risk stages
If a young patient, consider sperm banking for fertility preservation
If relapse occurs, consider high dose chemo with stem cell transplant
What is Non-Hodgkin Lymphoma (NHL)?
Lymphoma where 90% is from B lymphocytes
Higher incidence in patients who are immunodeficient
Peak incidence between 20-40
What are clinical features of NHL?
Diffuse, painless, persistent lymphadenopathy
Extranodal involvement
Associated with EBV
B symptoms (in intermediate and high grade disease)
Diagnostic studies for NHL?
Lymph node biopsy
PET
CT
How do you treat NHL?
Dependent on specific type and grade
Range of options: can either monitor or aggressively treat with chemo
Tumor lysis prophylaxis
Bone marrow transplant
What criteria is used to stage Hodgkin and non-Hodgkin lymphoma?
The Ann Arbor System
What is Multiple Myeloma?
Malignancy of plasma cells (proteins), which produce an abundance of monoclonal paraprotein (M protein). Unknown cause. Median age of diagnosis is 65.
What are plasma cells?
Cells in the bone marrow that are differentiated B cells. They produce antibodies to fight infection. In MM they crowd out other cells in the bone marrow
What are clinical features of MM?
CRAB:
HyperCalcemia
Renal disease
Anemia (pancytopenia)
Bone pain (Osteolysis with bone pain, Osteoporosis,
Pathologic fractures)

Recurrent infections
Paraprotein levels are increased
Diagnostic findings with MM?
Anemia with normal cell morphology
ROULEAUX FORMATION (RBCs that stacked like coins)
MONOCLONAL SPIKE on serum electrophoresis
BENCE JONES PROTEIN in urine
Lytic lesions on Xray of skeleton
BMP: Hypercalcemia, increased serum protein
Renal failure
What is the classic triad of MM?
Plasmacytosis
Bone lytic lesions
M-protein in serum/urine
How do you manage MM?
Chemo
Radiation
Bone marrow transplant
Hypercalcemia treatment

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