CPP&T Hematology

Terms	Definitions
What do hypersegmented neutrophils indicate?	Vitamin B12 / Folate deficiency
What do bite cells indicate?	G6PD deficiency
What do ringed sideroblasts indicate?	sideroblastic anemia
What do shistocyte or helmet cells indicate?	Microangiopathic anemia (DIC, TTP, traumatic hemolysis )
What do sickle cells indicate?	sickle cell anemia
What do spherocytes indicate?	hereditary spherocytosis, autoimmune hemolysis
What do teardrop cells in peripheral blood smear indicate?	bone marrow infiltration (i.e. myelofibrosis)
What do target cells in peripheral blood smear indicate?	HbC disease, aslenia, liver disease, thalassemia
What do howell-jolly bodies on peripheral blood smear indicate?	hyposplenia (asplenia also; occur b/c spleen doesn't RBC's like it usually would)
B12 deficiency anemia is usually caused by	malabsorption
Folate deficiency anemia is usually caused by	malnutrition
What is indicated in a microcytic anemia with central pallor?	Iron deficiency anemia (expect decreased ferritin and increased TIBC)
What is indicated in a megaloblastic anemia?	Vitamin B12 / folate deficiency
How are ferritin and TIBC (total iron binding capacity) altered in anemia of chronic disease?	Increased, decreased
In the anemia of chronice disease, would you expect iron levels in the bone marrow to be low or high?	High (think sickle cell, where body increases absorption of iron and you get iron overload)
What virus causes aplastic crisis in hereditary spherocytosis, sickle cell, etc?	Parvovirus b19
Which two anemias cause the "crew cut skull?"	sickle cell and thalassemia
What does an increase in unconjugated bilirubin indicate?	Hemolytic anemia
What does an increase in conjugated bilirubin indicate?	Hepatitis
Burton's lines	lead poisoning
Most common genetic cause of hypercoagubility?	Factor V Leiden (mutant Factor V cannot be degraded by protein C)
Reed-Sternberg Cells	Hodgkin's lyphoma
Fibrous/collagen bands, lacunar cells, and Reed-sternberg cells	nodular scelerosing hodgkin's lymphoma
"starry sky" lymph biopsy	Burkitt's lymphoma (non-hodgkin's)
What dangerous complication do we worry about post-treatment of Burkitt's lymphoma?	Acute lysis syndrome (lysis of tumor cells releases ions; causes acute renal failure; increased LDH and uric acid confirms dx)
Rouleaux formation in peripheral blood smear	multiple myeloma
Auer rods and myeloblasts in peripheral blood smear	acute myelogenous leukemia
Lymphocytosis with smudge cells	chronic lymphocytic leukemia
Late stage myeloproliferateive complication of CML	myelofibrosis
Mechanism of heparin	activates antithrombin (decreases thrombin and Xa)
What strange toxicity do we worry about with heparin?	Heparin Induced thrombocytopenia (immune rxn to platelets; results in platelet reduction
What serious side effect do we worry about with heparin?	Heparin induced thrombocytopenia
Which measure of clotting is increased in patients on warfarin?	PT (prothrombin time or extrinsic pathway)
Which measure of clotting is increased in patients on heparin?	PTT (partial thromboplastin time)
What would an episodic hemolytic anemia indicate?	G6PD deficiency (anemia triggered by oxidative stress like TMPSMX, antimalarial drugs, fava beans)
Which demographic gets ALL?	Children (CLL is more prevalent in adults)
What does cytoplasmic blebbing ("hand mirror cells") indicate?	Acute lymphoblastic leukemia
In which disorder would you expect to see osteolytic lesions, renail failure, anemia, and hypercalcemia?	Multiple myeloma
With which infection is Burkitt's lymphoma associated?	EBV
What condition does the "M spike" on serum protein electrophoresis diagnose?	Multiple myeloma
Which chromosomal translocation is associated with chronic myelogenous leukemia?	9:22 (Philadelphia chromosome, creating the BCR/ABL protein)
What do we worry about in advanced CML?	Blast crisis (development of AML or ALL)
Why myeloproliferative disorder results in increased RBC's?	polycythemia vera
With which disease are micromegakaryocytes associated?	Myelodysplastic syndrome
What blood smear abnormality would be seen with essential thrombocythemia?	Increased platelets
What gene is upregulated in Burkitt's lymphoma?	C-myc (usually from a t(8:14) translocation)
What is indicated in a unilateral lymphadenopathy that spreads in a chain fashion?	Hodgkin lymphoma
What toxicity (in addition to myelosuppression and mucositis) do we worry about with doxorubicin? (adriamycin)	cardiotoxicity (reduced LVEF)
What toxicity (in addition to myelosuppression and GI toxicity) do we worry about with methotrexate?	Renail failure (MTX crystallizes in the tubules; MTX is a folic acid inhibitor)
Which special toxicity do we worry about with taxanes?	Hypersensitivity reactions, stock-glove neuropathy
Which special toxicity do we worry about with vincas?	Neuropathy (vincristine)
Will LDH, AST, haptoglobin, and bilirubin increase or decrease in hemolytic states?	Increase, increase, decrease, increase
What is a special toxicity of cyclophosphamide?	Hemorrhagic cystitis (tx w/MESNA)
What is a special toxicity of bleomycin?	Pulmonary fibrosis
What is a special toxicity of cisplatin/carbaplatin?	nephrotoxicity
What causes eosinophilia? (acrynym used by FA is the answer)	NAACP (neoplastic, asthma, allergic processes, collagen vascular diseases, parasites)
What disease occurs because of a deficiency in factor VIII?	Hemophila A
What disease occurs because of a deficiency in factor IX?	Hemophila B
Factor II, VII, IX, X, protein C, and protein S can all be decreased because of a deficiency of what?	Vitamin K
A deficiency in what causes Bernard Soulier syndrome?	Bp1b
A deficiency in what causes Glanzmann's thrombasthenia?	GpIIB/IIIa
What does warfarin inhibit?	Vitamink K epoxide reductase (prevents Vitamin K from serving as cofactors of factor II, VII, IX, X, C, S)
What does heparin affect?	Antithrombin (inactivates factor II, VII, IX, X, XI)
What are the microcytic, hypochormic anemias?	Iron deficiency, alpha thalassemia, beta thalassemia, lead poisoning, sideroblastic anemia (MCV < 80)
In what disease would we expect to see increased HbA2?	B-thalassemia (B chain is absent/underproduced)
How will methylmalonic acid be affected by B12 deficiency?	Increased (Normal in folate deficiency)
How does iron change in intrinsic anemia?	Decreased (increased in extrinsic anemia b/c it is salvaged AND uptake is increased)
By what mechanism does kidney disease cause anemia?	Decreased hematopoiesis (kidney necessary to release erythropoietin, so w/out kidney you get less RBC production)
What does a positive osmotic fragility test indicate?	Hereditary spherocytosis
What inheritance pattern does G6PD deficiency follow?	X-linked (decreased glutathione → increased RBC susceptibility to oxidant stress)
What causes paroxysmal nocturnal hemoglobinuria?	Compliment-mediated RBC lysis (increased urine hemosiderin in labs)
How are serum iron, transferrin (TIBC), and ferritin affected in iron deficiency anemia?	Decreased, increased, decreased
What does an increased bleeding time in the presence of normal PT and PTT indicate?	Platelet disorder (Bernard soulier, glanzmann'sthrombasthenia, ITP, TTP → expect low platelets)
In which disease does an antibody response to platelets cause decreased platelet survival and increased bleeding time?	Idiopathic thrombocytopenic purpura
In which disease do large vWF multimers cause increased platelet aggregation and thrombosis? (leads to decreased platelet survival)	thrombotic thrombocytopenic purpura (expect shistocytes and increased LDH in labs; triad of neurologic symptoms + thrombocytopenia and microangiopathic hemolytic anemia)
What tests the function of factors I, II, V, VII, and X?	prothrombin time (extrinsic pathway!)
What tests the function of everything except VII and XIII?	Partial thromboplastin time (intrinsic pathway)
In what disorder would you expect bleeding into joints, hemorrhages, easy bruising, increased PT and/or PTT?	Hemophila A or B
What's the most common cause of inherited hypercoagulability?	Factor V Leiden (40-50% of cases; mutant factor V cannot be degraded by factor V )
What does a lymphoma that travels from node to nearby node indicate?	Hodgkin's lymphoma
What type of lymphoma would we expect if the pt. presented with low grade fever, night sweats, and weight loss?	Hodgkin's lymphoma (these are the so-called "B-signs")
If a patient with Burkitt's lymphoma stops producing urine soon after treatment begins, what would we suspect?	Acute tumor lysis syndrome (confirmed by tests indicating increased LDH and uric acid)
What does an 8:14 translocation indicate?	Burkitt's lymphoma (c-myc gene upregulated behind Igg promoter)
What does a 14:18 translocation indicate?	Follicular lymphoma (bcl-2 expression)
In a lymphoblastic leukemia in a young child, what disease would we expect?	ALL
In a lymphoblastic leukemia in an older person, what would we expect?	CLL
What does a 9:22 translocation indicate?	CML (Philadelphia chromosome)
What does a 15;17 translocation idicate?	AML (M3 type)
What does an 11:14 translocation indicate?	Mantle cell lymphoma
In what disorder would we expect proliferation of RBCs, WBCs, and platelets?	Polycythemia vera (abnormal hematopoietic stem cell clones)
What anticoagulant can be prescribed during pregnancy?	Heparin (warfarin crosses the placental barrier)
How do we reverse heparin?	Protamine sulfate (binds negatively charged heparin molecules and heparin has short action anyway)
How do we reverse warfarin?	IV Vitamin K, fresh frozen plasma
How do we monitor heparin?	PTT (intrinsic pathway!)
How do we monitor warfarin?	PT/INR (extrinsic pathway)