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Hemeonc

Instructions:

Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
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1. t(8;14) c - myc gene
Burkitt lymphoma
Mantle cell - older males
Extravascular hemolysis
Factor V Leiden

2. What substance is Fe added to to yield heme
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Nl - up - nl
Dendritic cells?
Protoporphyrin

3. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Low in CML
HIV or immunosupression
Signs and sx of aplastic anemia
DIC

4. RBCs damagaed from passing through obstructed - narrowed vessel lumina
CML to AML or All
MAHA
20 to 40
TXA2 - dec blood flow - inc platelet aggregation

5. Does CML have a JAK2 mut
Hb Barts - gamma4 in defect in all 4 alpha genes
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
No
VWF and fibrinogen

6. What state is commonly associated with nonHod lymphoma
Porphyrias
Blasts > 5%
HIV or immunosupression
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

7. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Beta chain underproduced - asymptomatic - heterozygote
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Idiopathic thrombocytopenia (ITP)

8. Which drugs can cause macrocytic anemia?
Lymphoma
5- FU - AZT - hydroxyurea
Fc
Lysine for glutamate at position 6

9. What chromosomal translocation is associated with a better prognosis in All
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
120 days
T(12;21)
No platelet clumping

10. What percentage if WBCs are polys?
CML to AML or All
Petechiae
Exposed collagen upon endothelial damage
40 to 75

11. What are the anti aggregation factors?
PGI2 - NO inc blood flow - dec platelet aggregation
Both alpha and beta thal
B symptoms - fever night sweats - weight loss
No antigen - both antibodies

12. What is the coombs test results in AIHA?
Pos
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
B antigena and A antibodes
Dec plasma volume

13. What causes the jaundice in extravascular hemolysis
Inc UCB
Low in CML
Dec synthesis of factors 1972 - protein C/S
Leukemoid reaction

14. What is the affected enzyme in acute intermittment porphyria
Porphobilinogen - delta ALA - uroporphyrin
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Collagen - BM - activated platelets
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

15. What do auer rods stain with
Peroxidase
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
G6PD
M3 AML (acute promyelocytic leukemia)

16. What is the pathogenesis of sickle cell
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
120 days
VWD
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

17. Macro - ovalocyte
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Sickle cell anemia
Down - up - down

18. What is the characteristic histologic finding in Hodgkin Lymphoma
Hairy cell leukemia
Reed - sternberg cells
Tissue factor converst the VII and VIIa
Fc

19. Upregulated growth of leukocytes in bone marro
Ticlopidine/clopidogrel
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Leukemia
Down - down - up

20. normal RBCs added to patient's serum
Indirect coombs - agglutinate if serum anti RBC surface Ig
Beta thal
EBV
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

21. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
IgG - Warm is GREAT
IFN gama
Dec plasma volume

22. How is beta thal minor dx
Inc HbA2 on electrophoresis
Alpha thal - asian and african american
Liver disease - abetalipoproteinemia - acntho = spiny
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

23. Blood type B
B antigena and A antibodes
Macrohemorrhage - hemarthroses - easy bruising
Dec synthesis of factors 1972 - protein C/S
MHC II and Fc receptors

24. Which pathway and factors are tested in the PTT test
Factor V resistant to activated protein C's inhibition
Howell Jolly bodies
Intrinsic - all factors except - VII - XIII
Plasma cell

25. CD5+ - poor prognosis - t(11;14)
Collagen - BM - activated platelets
Mantle cell - older males
T(9;22) bcr abl
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

26. What are the likely exposures of kids and adults for lead poisoning
Kids= exposure to lead paint - adults = battery - ammunition factory
Porphobilinogen - delta ALA - uroporphyrin
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Anti Rh

27. What is the effect of ACE on bradykinin
Protoporphyrin
Multiple myeloma
Inactivates it
G6PD

28. What is appropriate absolute polycythemia
Ringed sideronblasts with iron laden mitochondria
Reed - sternberg cells
Inc RBC - dec O2 sat - inc EPO
ATIII def

29. What is the tx for vWD
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Lysosomes
Valine for glutamate
Def in factor IX

30. What substance accumulates in porphyria cutanea
Extrinsic - I - II - V - VII and X
Uroporphyrin (tea colored urine)
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
MAHA

31. Serum iron - transferrin - ferritin lab values for iron def anemia
CML to AML or All
Down - up - down
Orotic aciduria
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

32. Fibrotic obliteration of bone marow with teardrop cells

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33. What are some classic examples of extravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell
Tissue
Uroporphyrin (tea colored urine)
VWF and fibrinogen

34. Sickle cell
All trans retinoic acid
Factor V resistant to activated protein C's inhibition
Sickle cell anemia
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

35. Mutation in 3' untranslated region associated with venous clots
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Intravascular hemolysis
Prothrombin gene mutation

36. Fava beans - sufla drugs - infectinos
They bind vWF via GpIb
Examples of oxidative stress
Kids= exposure to lead paint - adults = battery - ammunition factory
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

37. How are the nucleus and the cytoplasm characterized for lymphocytes
Beta thal
Round densly staining nucleus with a small amount of pale cytoplasm
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

38. What substances are released in mast cell degranulation
Prothrombin gene mutation
Histamine - heparin - and eosinohil chemotactic factors
Dec synthesis of factors 1972 - protein C/S
Pyruvate kinase def - extravascular

39. What is hemophilia B?
Collagen - BM - activated platelets
Def in factor IX
Nl PT - elevated PTT - intrinsic pathway defect
Hairy cell leukemia

40. What do platelets interact with to form a hemostatic plug
5- FU - AZT - hydroxyurea
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Fe def - thal - pb poison - sideroblastic anemia
Fibrinogen

41. What do the platelets bind? What is the step called
Pos
They bind vWF via GpIb
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell

42. Who has more severe disease - HbSS or HbSC
Mycosis fundgoides/Sezary syndrome
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Collagen - BM - activated platelets
HbSS

43. Target cell

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44. What is the Ddx for a normocytic - normochromic anemia?
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Liver disease - abetalipoproteinemia - acntho = spiny
Mantle cell - older males
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

45. What is hemophiliia A
Dendritic cells?
Deficiency in factor VIII
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
They bind vWF via GpIb

46. What finding you do you see in patients after splenectomy
Howell Jolly bodies
ADP and Ca
Ringed sideronblasts with iron laden mitochondria
CLL (SLL without the peripheral lymphocytosis

47. discrete tumor masses arising from lymph nodes
TXA2 - dec blood flow - inc platelet aggregation
Lymphoma
Extravascular hemolysis
B12 in fatty acid pathways leads to subacute combined degeneration

48. What are the extrinsic hemolytic normocytic anemias?
...
Nl PT - elevated PTT - intrinsic pathway defect
CML to AML or All
Indirect coombs - agglutinate if serum anti RBC surface Ig

49. How does the therapy for M3 vairant work?
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Induces differentiation of myeloblasts
All
Lymphoma

50. Serum iron - transferrin - ferritin lab values for hemochromatosis
Diffuse Large b cell lymphoma
Up - down - up
Hereditary spherocytosis - autoimmune hemolysis
TRAP (tartrate resistant acid phosphatase