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Hemeonc

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Answer 50 questions in 15 minutes.
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1. Deficiency in GpIb
Bernard soulier
Birbeck granules
Paraprotein spike - monoclonal protein
CML

2. What is the philadelphia chromosome
Bernard soulier
Extrinsic - I - II - V - VII and X
T(9;22) bcr abl
Von Willebrand's disease

3. What is the receptor for vWF
8-10 days
Gp1b
Inducers of primary antibody response
Aspirin

4. What does CRAB stand for in multiple myeloma
Varying shapes
Dec synthesis of factors 1972 - protein C/S
Diffuse Large b cell lymphoma
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

5. Target cell

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6. Is G6PD intravascular or extravascular
...
Signs and sx of aplastic anemia
Intravascular
Lung disease - congenital heart diseaes - and high altitude

7. What does the blood smear show in glanzmann's?
Sideroblastic anemia
Alpha thal - asian and african american
DDAVP (desmopressin) which releases stored vWF stored in endothelium
No platelet clumping

8. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Nl - up - nl
Dendritic cells?
Degrades fibrin mesh and converts C3 to C3a
Petechiae

9. What are some classic examples of extravascular hemolysis
Hereditary spherocytosis - G6PD - sickle cell
Induces differentiation of myeloblasts
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Multiple myeloma

10. Serum iron - transferrin - ferritin lab values for hemochromatosis
VWF and fibrinogen
M3 AML (acute promyelocytic leukemia)
Up - down - up
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

11. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Up - down - nl
Collagen - BM - activated platelets
Lung disease - congenital heart diseaes - and high altitude
Malaria - Babesia

12. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Down - down - up
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -
Essential thrombocytosis
Orotic aciduria

13. bcl -2 t(14;18) adults
Follicular lymphoma - indolent course
Anemia of chronic disease - Aplastic anemia - kidney disease
Common a few days after oxidative stress in a pt with G6PD
HIV or immunosupression

14. What is the pathogenesis of aplastic anemia with kidney disease
Decrease EPO
Aspirin
Lacunar in nodular sclerosing variant
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

15. Which factor does vWF carry/protect
B12 def
MGUS - monoclonal gammopathy of undetermined significance
VIII
SLE - CLL - alpha methyldopa

16. What are the anti aggregation factors?
PGI2 - NO inc blood flow - dec platelet aggregation
Examples of oxidative stress
Mantle cell lymphoma
Africa = Jaw lesion - US = pelvis or abdomen

17. What CD molecules are on RS cells
Thrombotic thrombocytopenic purpura
Fibrinogen
Ticlopidine/clopidogrel
CD15 and CD30 pos

18. What do plasma cells do
Blod - tissue - MACS
Produce antibody - lots of RER and golgi
Beta 4
Leukemia

19. drug that inhibits ADP induced expression of GpIIb/IIIa
Collagen - BM - activated platelets
Ticlopidine/clopidogrel
Kids= exposure to lead paint - adults = battery - ammunition factory
Sideroblastic anemia

20. What converts plasminogen to plasm and What does plasmin do?
Low O2 in papilla; can also get microhematuria from medullary infarcts
Folate def
TPA - cleavage of fibrin mesh
Up - down - nl

21. Fibrotic obliteration of bone marow with teardrop cells

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22. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Hb Barts - gamma4 in defect in all 4 alpha genes
Peroxidase
Folate def
DDAVP (desmopressin) which releases stored vWF stored in endothelium

23. What is appropriate absolute polycythemia
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Multiple myeloma
Round densly staining nucleus with a small amount of pale cytoplasm
Inc RBC - dec O2 sat - inc EPO

24. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Bone marrow infiltration - myelofibrosis
Incactivates II - VII - IX - X - XI - XII
Contiguous
Factor V Leiden

25. What is the Ddx for a normocytic - normochromic anemia?
AML
Intravascular
MHC II and Fc receptors
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

26. What is a metabolic disorder tht can cause macrocytic anemia?
Orotic aciduria
Dendritic cells?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Decrease EPO

27. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Complications of sickle cell anemia
VWD
Examples of oxidative stress
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

28. What percentage if WBCs are polys?
Paroxysmal nocturnal hemoglobinuria
Epoxide reductase - warfarin inhibits
Nl - up - nl
40 to 75

29. Conditions of defective heme synthesis leading to accumulation of heme precurors
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Porphyrias
B symptoms - fever night sweats - weight loss
Factor V resistant to activated protein C's inhibition

30. What causes the physiologic chloride shift and What does the chloride shift do?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Kids= exposure to lead paint - adults = battery - ammunition factory
ADP and Ca
Intrinsic - all factors except - VII - XIII

31. What the alpha granules contain in platelets?
Nodular sclerosing
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Protoporphyrin
VWF and fibrinogen

32. What is a blast crisis
They bind vWF via GpIb
Spleen
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
CML to AML or All

33. Which immunoglobulin is involved in warm agglutination?
Inc RBC - dec O2 sat - inc EPO
Africa = Jaw lesion - US = pelvis or abdomen
IgG - Warm is GREAT
Sideroblastic anemia

34. Basophilica stippling
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Inc vasodiltion - inc perm - inc pain
Dec plasma volume
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

35. What virus can cause an aplastic crisis in pts with HS?
Hereditary spherocytosis - autoimmune hemolysis
Parvovirus
No lytic bone lesions in WM
Paroxysmal nocturnal hemoglobinuria

36. What percentage of WBCs are basophils - and What is found in their basophilic granules?
Parvovirus
ADP and Ca
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

37. Schisotcyte - helmet cell
Porphobilinogen - delta ALA - uroporphyrin
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
DIC - TTP/HUS - traumatic hemolysis
Fc

38. What is the accumulated substance in lead poisoning
90% anearobically from glucose to lactate - 10% from HMP shunt
Varying sizes
Protoporphyrin (blood)
Dendritic cells?

39. anisocytosis
Antigen - antibody
Inc lymphoctes - and less RS cells
Bite cells and Heinz bodies
Varying sizes

40. Drug that inhbits the GpIIb/IIIa directly
Malaria - Babesia
Abciximab
Histaminase and arylsulfatase
EBV

41. What is the enzyme defect in Porphyria cutanea tarda
5- FU - AZT - hydroxyurea
Hodgkin
Diffuse Large b cell lymphoma
Uroporphyrinogen decarboxylase

42. What is the mutation in HbS
Dec synthesis of factors 1972 - protein C/S
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Nl PT - elevated PTT - intrinsic pathway defect
Valine for glutamate

43. Which drugs can cause macrocytic anemia?
5- FU - AZT - hydroxyurea
Multiple myeloma
GpIIb/IIIa
Sideroblastic anemia

44. hypocellular bone marrown tih fatty infiltration
Hairy cell leukemia
Lacunar in nodular sclerosing variant
Aplastic anemia - pancytopenia
Dendritic cells?

45. What do you see a starry sky appearance in Burkitt
Schistocytes and inc LDH
Low in CML
Sheets of lymphocytes interspersed with macs
Inc vasodiltion - inc perm - inc pain

46. What vitamin is a cofactor for the first step of heme synthesis
Hairy cell leukemia
Protoporphyrin (blood)
CNS and testis
B6

47. Blood type B
Intravascular hemolysis
B antigena and A antibodes
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Degrades fibrin mesh and converts C3 to C3a

48. What is relative polycythemia
Schistocytes and inc LDH
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Dec plasma volume
Ticlopidine/clopidogrel

49. What do they express on their surface?
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Varying shapes
MHC II and Fc receptors
Abciximab

50. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Hereditary spherocytosis
B6 therapy (pyrodixine)
Hodgkin
GpIIb/IIIa

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