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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. What activates the intrinsic pathway?
Hereditary spherocytosis - G6PD - sickle cell
Multiple myeloma
Helminth infections major basic protein
Collagen - BM - activated platelets

2. What is the age breakdown for hodgkins
Signs and sx of aplastic anemia
Lacunar in nodular sclerosing variant
Bimodal - men>women - except for nodular sclerosing type
CLL (SLL without the peripheral lymphocytosis

3. What is the receptor for vWF
Valine for glutamate
All trans retinoic acid
Gp1b
Porphobilinogen - delta ALA - uroporphyrin

4. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Signs and sx of aplastic anemia
B cells
Glucose and heme - which inhibit ALA synthase
Beta thal

5. Schisotcyte - helmet cell
Low in CML
DIC - TTP/HUS - traumatic hemolysis
Fibrinogen
Pyruvate kinase def - extravascular

6. iron deficiency anemia - esophageal web - atrophic glossitis
Hereditary spherocytosis
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Plummer - vinson syndrome
DIC - TTP/HUS - traumatic hemolysis

7. What does antithrombin do and What activates it?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Down - up - down
Histamine - heparin - and eosinohil chemotactic factors
Helps platelts adhere to endothelium

8. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Aplastic anemia - pancytopenia
Bleeding - malnutrition/absorption - inc demand pregs
Peroxidase
VWD

9. Which cell is neoplastic in multiple myeloma
Hairy cell leukemia
T(9;22) bcr abl
Plasma cell
Rhogam - Rh antigen immunoglobulin

10. When is the peak incidence for nonHod lymphoma
Ringed sideronblasts with iron laden mitochondria
Sideroblastic anemia
Pyruvate kinase def - extravascular
20 to 40

11. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
Inc vasodiltion - inc perm - inc pain
IFN gama
Imatinib - anti bcr abl
Heinz bodies - seen in alpha thal and G6PD

12. inc LDH - jaundice
Macrohemorrhage - hemarthroses - easy bruising
Plasma cell
Extravascular hemolysis
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

13. Wilm's tumor - RCC - HCC - hydronephrosis
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Def in factor IX
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Dec

14. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Inc
Bite cells and Heinz bodies
Nl - up - nl
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

15. Deficiency in vWF

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16. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Histaminase and arylsulfatase
T(9;22) bcr abl

17. What are the azuraphilic granules in PMNs
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Lysosomes
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

18. What state is commonly associated with nonHod lymphoma
HIV or immunosupression
EBV
Aspirin
Dec plasma volume

19. Where are mast cells found
Inc UCB
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 -
8-10 days
Tissue

20. What is hemophilia B?
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Def in factor IX

21. What is the pathogenesis of TTP?
Malaria - Babesia
Inc HbA2 on electrophoresis
Histaminase and arylsulfatase
Inc large vWF multimers - inc platelet aggregation and thrombosis

22. What is the most common nonhodgkin lymphoma
Diffuse Large b cell lymphoma
Varying sizes
B12/folate def
Inc UCB

23. Serum iron - transferrin - ferritin lab values for hemochromatosis
Hereditary elloptocytosis
Up - down - up
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Burkitt lymphoma

24. t(11;14)
Direct coombs - agglutinate if RBCs are coated with Ig
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Mantle cell lymphoma
Abciximab

25. Which factor does vWF carry/protect
Complications of sickle cell anemia
VIII
Up - down - up
Protein C or S deficiency

26. What are the neuro sx of B12 def?
Examples of oxidative stress
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Deficiency in factor VIII
DIC

27. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
All
Extravascular
Dec synthesis of factors 1972 - protein C/S

28. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Protein C or S deficiency
B6
Collagen - BM - activated platelets
Tissue

29. Who has more severe disease - HbSS or HbSC
Pos
HbSS
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Factor V resistant to activated protein C's inhibition

30. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Birbeck granules
Lysosomes
Leukemoid reaction
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

31. What is the characteristic histologic finding in Hodgkin Lymphoma
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Hereditary spherocytosis - autoimmune hemolysis
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Reed - sternberg cells

32. What do labs show in ITP?
Inc vasodiltion - inc perm - inc pain
Blasts > 5%
Rhogam - Rh antigen immunoglobulin
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

33. What substance accumulates in porphyria cutanea
Multiple myeloma
Examples of oxidative stress
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Uroporphyrin (tea colored urine)

34. what proteins can be defective in HS?
Histaminase and arylsulfatase
Extrinsic - I - II - V - VII and X
Ankryin - band 4.1 or spectrin
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

35. discrete tumor masses arising from lymph nodes
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Mantle cell - older males
Lymphoma
Folate def

36. What is the purpose of the fibrin mesh
Sideroblastic anemia
Blistering cutaneous photosens - most common porphyria
Acts to stabilize platelet plug
PGI2 - NO inc blood flow - dec platelet aggregation

37. What is the philadelphia chromosome
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Off center nuclues - clock face chromatin
T(9;22) bcr abl

38. How does the therapy for M3 vairant work?
Ankryin - band 4.1 or spectrin
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
B12/folate def
Induces differentiation of myeloblasts

39. What are the main associations with multiple myeloma?
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Helps platelts adhere to endothelium
MAHA
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

40. drug that inhibits ADP induced expression of GpIIb/IIIa
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
SLE - CLL - alpha methyldopa
Ticlopidine/clopidogrel
Hereditary spherocytosis - G6PD - sickle cell

41. What happens in beta thal major?
Low in CML
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
CD15 and CD30 pos
No lytic bone lesions in WM

42. What is relative polycythemia
Factor V Leiden
Dec plasma volume
Down - up - down
Howell - Jolly bodies - hypo/asplenia

43. What is the therapy for the M3 variant?
Anemia of chronic disease - Aplastic anemia - kidney disease
Histaminase and arylsulfatase
Produce antibody - lots of RER and golgi
All trans retinoic acid

44. What percentage if WBCs are polys?
Down - up - down
Thrombotic thrombocytopenic purpura
40 to 75
MAHA

45. What causes renal papillary necrosis in sickle cell
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
VIII
Low O2 in papilla; can also get microhematuria from medullary infarcts
Protein C or S deficiency

46. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Histamine - heparin - and eosinohil chemotactic factors
Hodgkin
Folate def

47. What vitamin is a cofactor for the first step of heme synthesis
B6
Bimodal - men>women - except for nodular sclerosing type
ADP and Ca
G6PD

48. What is the mutation in HbC
Lysine for glutamate at position 6
Megakaryocytes
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

49. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Megakaryocytes
Her next Rh+ fetus
M3 AML (acute promyelocytic leukemia)
GpIIb/IIIa

50. t(15;17)
B cells
Schistocytes - helmet cells
Inactivates it
M3 AML (acute promyelocytic leukemia)