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Hemeonc

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Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
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1. CD5+ - poor prognosis - t(11;14)
Glucose and heme - which inhibit ALA synthase
HbSS
1 to 6
Mantle cell - older males

2. What is the effect of ACE on bradykinin
T(9;22) bcr abl
Inactivates it
Varying shapes
Inc HbF and dec HbS

3. What is relative polycythemia
Glucose and heme - which inhibit ALA synthase
Extravascular
Intravascular hemolysis
Dec plasma volume

4. What are the variants of the RS cells
Sideroblastic anemia
Lacunar in nodular sclerosing variant
Dec
Eleveated PT - PTT

5. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Incactivates II - VII - IX - X - XI - XII
VWD
Complications of sickle cell anemia
Inc large vWF multimers - inc platelet aggregation and thrombosis

6. What is hemophilia B?
Reed - sternberg cells
Mantle cell - older males
Def in factor IX
Both alpha and beta thal

7. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Decrease EPO
Histaminase and arylsulfatase
B12 def
Fc

8. What is the treatment for sideroblastic anemia
VWD
B6 therapy (pyrodixine)
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Sheets of lymphocytes interspersed with macs

9. What is the receptor for vWF
Gp1b
Factor V Leiden
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Anti Rh

10. What do you see in peripheral smear in a pt with G6PD?
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Diffuse Large b cell lymphoma
Bite cells and Heinz bodies
Tissue factor converst the VII and VIIa

11. What causes renal papillary necrosis in sickle cell
Leukemia
Porphobilinogen - delta ALA - uroporphyrin
Sideroblastic anemia
Low O2 in papilla; can also get microhematuria from medullary infarcts

12. What is the Ddx for a normocytic - normochromic anemia?
Aspirin
Down - down - up
Orotic aciduria
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

13. Serum iron - transferrin - ferritin lab values for pregs - OCP use
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
T(12;21)
Nl - up - nl
VWF carries/protects factor VIII

14. How do platelet disorders present?
Paroxysmal nocturnal hemoglobinuria
VIII
Leukemia
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

15. What do labs show in DIC?
Langerhans cells
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

16. Megakaryocytosis
Anti Rh
Malaria - Babesia
EBV
Essential thrombocytosis

17. Where does All spread
VIII
Prothrombin gene mutation
CNS and testis
Burkitt lymphoma

18. Schisotcyte - helmet cell
Bone marrow - thymus - blood (80% of circulating lymphos are T)
DIC - TTP/HUS - traumatic hemolysis
All trans retinoic acid
Ewing sarcoma

19. bcl -2 t(14;18) adults
Follicular lymphoma - indolent course
Malaria - Babesia
Macrohemorrhage - hemarthroses - easy bruising
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

20. What is the characteristic histologic finding in Hodgkin Lymphoma
Tissue factor converst the VII and VIIa
Reed - sternberg cells
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
DDAVP (desmopressin) which releases stored vWF stored in endothelium

21. Which pathway and factorrs are tested by the PT coag test
Alpha thal - asian and african american
Extrinsic - I - II - V - VII and X
...
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

22. What does decreasing heme do to ALA synthase activity?
Inc
B6
Macrohemorrhage - hemarthroses - easy bruising
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)

23. X linked - dec glutathione inc RBC susceptibility to oxidative stress
G6PD
TXA2 - dec blood flow - inc platelet aggregation
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Fc

24. What are the azuraphilic granules in PMNs
Factor V resistant to activated protein C's inhibition
Dendritic cells?
Lysosomes
Bone marrow - thymus - blood (80% of circulating lymphos are T)

25. What do labs show in ITP?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Produce antibody - lots of RER and golgi
Macrohemorrhage - hemarthroses - easy bruising
Aplastic anemia - pancytopenia

26. What activates protein C and What does activated protein C do?
Lysosomes
M3 AML (acute promyelocytic leukemia)
Protein S - cleaves and invactivates Va and VIIIa
Hairy cell leukemia

27. Is G6PD intravascular or extravascular
40 to 50
Cromolyn sodium
Idiopathic thrombocytopenia (ITP)
Intravascular

28. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Folate def
G6PD
Sideroblastic anemia
Ferrocheletase and ALA dehydrogenase

29. What does ADP do?
Lacunar in nodular sclerosing variant
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Helps platelts adhere to endothelium
Ringed sideronblasts with iron laden mitochondria

30. Conditions of defective heme synthesis leading to accumulation of heme precurors
Complications of sickle cell anemia
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Porphyrias
Plasma cell

31. anti - Ig antibody added to patients RBCs;
Direct coombs - agglutinate if RBCs are coated with Ig
Folate def
Positive osmootic fragility test and splenectomy
Schistocytes - helmet cells

32. What finding you do you see in patients after splenectomy
Africa = Jaw lesion - US = pelvis or abdomen
Howell Jolly bodies
ADP and Ca
Folate def

33. What condition can result from treating AML M3 from the release of the Auer rods
Reed - sternberg cells
DIC
Paroxysmal nocturnal hemoglobinuria
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

34. hypocellular bone marrown tih fatty infiltration
CNS and testis
Histamine - heparin - and eosinohil chemotactic factors
Aplastic anemia - pancytopenia
Antigen - antibody

35. What are the four levels of alpha thal?
Both alpha and beta thal
Ticlopidine/clopidogrel
CML
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

36. What cell is primarily involved in non Hod lymph
B cells
Signs and sx of aplastic anemia
Leukemia
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

37. What is the age breakdown for hodgkins
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Africa = Jaw lesion - US = pelvis or abdomen
CLL (SLL without the peripheral lymphocytosis
Bimodal - men>women - except for nodular sclerosing type

38. Is HS extravascular or intravascular?
Extravascular
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
B antigena and A antibodes
T(9;22) bcr abl

39. Eosinophils are highly phagocytic For what kind of complex?
VWF carries/protects factor VIII
Down - up - down
Antigen - antibody
Idiopathic thrombocytopenia (ITP)

40. What does Vit K deficiency cause?
Nodular sclerosing
B antigena and A antibodes
Dec synthesis of factors 1972 - protein C/S
Lacunar in nodular sclerosing variant

41. What is the philadelphia chromosome
Paroxysmal nocturnal hemoglobinuria
T(9;22) bcr abl
Mycosis fundgoides/Sezary syndrome
Low in CML

42. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Valine for glutamate
Tissue
Her next Rh+ fetus
...

43. What does hairy cell leukemia stain with
Indirect coombs - agglutinate if serum anti RBC surface Ig
Paraprotein spike - monoclonal protein
Plasma cell
TRAP (tartrate resistant acid phosphatase

44. What the alpha granules contain in platelets?
Acts to stabilize platelet plug
Petechiae
VWF and fibrinogen
Schistocytes - helmet cells

45. universal donor
Folate def
Inactivates it
No antigen - both antibodies
Hereditary elloptocytosis

46. Plasma cell neoplasm
40 to 50
VWF carries/protects factor VIII
Multiple myeloma
Anti Rh

47. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
DIC
Mantle cell lymphoma
Indirect coombs - agglutinate if serum anti RBC surface Ig

48. Causes of iron def?
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Bleeding - malnutrition/absorption - inc demand pregs
Varying sizes
Heinz bodies - seen in alpha thal and G6PD

49. discrete tumor masses arising from lymph nodes
Lymphoma
Up - down - nl
Rhogam - Rh antigen immunoglobulin
Pos

50. Elliptocyte
No lytic bone lesions in WM
Lysine for glutamate at position 6
Orotic aciduria
Hereditary elloptocytosis