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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. What is the receptor for fibrinogen?
Beta thal
GpIIb/IIIa
B12 in fatty acid pathways leads to subacute combined degeneration
CML

2. poikilocytosis
Varying shapes
Low in CML
Yes - via MHC II
Lung disease - congenital heart diseaes - and high altitude

3. What is hemophilia B?
Inc RBC - dec O2 sat - inc EPO
Aplastic anemia - pancytopenia
Heinz bodies - seen in alpha thal and G6PD
Def in factor IX

4. What virus can cause an aplastic crisis in pts with HS?
Inducers of primary antibody response
Parvovirus
Multiple myeloma
Hereditary elloptocytosis

5. Basophilic nuclear remnants fonud in RBCs
SLE - CLL - alpha methyldopa
Howell - Jolly bodies - hypo/asplenia
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
40 to 50

6. What are the neuro sx of B12 def?
Multiple myeloma
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
ADP and Ca

7. What is the treatment for sideroblastic anemia
B6 therapy (pyrodixine)
Fibrinogen
No antigen - both antibodies
Extravascular

8. Can B cells function as APCs?
Inc HbF and dec HbS
IFN gama
Yes - via MHC II
Inc

9. Where are mast cells found
Eleveated PT - PTT
Africa = Jaw lesion - US = pelvis or abdomen
Tissue
No platelet clumping

10. What is the Ddx for nonhemolytic normocytic anemia
Idiopathic thrombocytopenia (ITP)
Anemia of chronic disease - Aplastic anemia - kidney disease
Mantle cell lymphoma
Lacunar in nodular sclerosing variant

11. What is a blast crisis
T(12;21)
Intravascular hemolysis
B12/folate def
CML to AML or All

12. dec haptoglobin - inc LDH - hemoglobin in urine (paroxysmal nocturnal hemoglobinuria - mechanical destruction as in aortic stenosis or a prosthetic valve
Intravascular hemolysis
Plummer - vinson syndrome
40 to 50
Factor V Leiden

13. Who has more severe disease - HbSS or HbSC
HbSS
Down - up - down
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Porphyrias

14. mother's antibodies attack fetal RBCs
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
...
Blasts > 5%
Lung disease - congenital heart diseaes - and high altitude

15. Which cell is neoplastic in multiple myeloma
40 to 75
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Plasma cell
Hereditary spherocytosis

16. t(11;14)
Collagen - BM - activated platelets
Nl - up - nl
Blistering cutaneous photosens - most common porphyria
Mantle cell lymphoma

17. How does vWD cause elevated PTT?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
VWF carries/protects factor VIII
Nodular sclerosing
Blasts > 5%

18. What is the result of thrombocytopenia or platelet dysfunction?
Petechiae
Fibrinogen
TXA2 - dec blood flow - inc platelet aggregation
Blood

19. What is the characteristic histologic finding in Hodgkin Lymphoma
B antigena and A antibodes
Reed - sternberg cells
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Intravascular

20. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Factor V Leiden
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Sickle cell
Uroporphyrinogen decarboxylase

21. What is makes a leukemia acute
Blasts > 5%
Petechiae
Glycine and succinyl - coa
Mycosis fundgoides/Sezary syndrome

22. What do the dense granules contain in platelets
Uroporphyrinogen decarboxylase
Blasts > 5%
ADP and Ca
Paraprotein spike - monoclonal protein

23. What do you see in peripheral smear in a pt with G6PD?
Leukemia
Bite cells and Heinz bodies
Von Willebrand's disease
Protein C or S deficiency

24. Ringed sideroblasts
HIV or immunosupression
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Sideroblastic anemia
CML

25. When is the peak incidence for nonHod lymphoma
20 to 40
All
40 to 50
EBV

26. bcl -2 t(14;18) adults
Fe def - thal - pb poison - sideroblastic anemia
Follicular lymphoma - indolent course
No antigen - both antibodies
B12 in fatty acid pathways leads to subacute combined degeneration

27. Bite cell
MGUS - monoclonal gammopathy of undetermined significance
Extravascular hemolysis
G6PD
Decrease EPO

28. Bone pain plus anemia
Petechiae
Hereditary spherocytosis - autoimmune hemolysis
Langerhans cell histiocytosis
Multiple myeloma

29. Deficiency in GpIb
Bernard soulier
Both alpha and beta thal
Ewing sarcoma
Reed - sternberg cells

30. Serum iron - transferrin - ferritin lab values for hemochromatosis
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Up - down - up
Fc
Imatinib - anti bcr abl

31. What do you see in vit K def
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Eleveated PT - PTT
B12/folate def
Def in factor IX

32. adults - auer rods - inc circulating myeblasts on peripheral smear
Nl PT - elevated PTT - intrinsic pathway defect
Inc lymphoctes - and less RS cells
MAHA
AML

33. What does ADP do?
Von Willebrand's disease
Helps platelts adhere to endothelium
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
AB - no antibodies

34. What are the anti aggregation factors?
PGI2 - NO inc blood flow - dec platelet aggregation
2 to 10
Splicing sites and promotor sequences
Low O2 in papilla; can also get microhematuria from medullary infarcts

35. What is the purpose of the fibrin mesh
Extrinsic - I - II - V - VII and X
1 to 6
Intravascular
Acts to stabilize platelet plug

36. What does increasing heme do to ALA synthase activity
Low O2 in papilla; can also get microhematuria from medullary infarcts
2 to 10
Dec
DIC

37. What are the etiologies of folate def
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Hereditary spherocytosis
Anti Rh

38. What do they express on their surface?
Epoxide reductase - warfarin inhibits
Orotic aciduria
MHC II and Fc receptors
Gp1b

39. What state is commonly associated with nonHod lymphoma
CML
HIV or immunosupression
All trans retinoic acid
Splicing sites and promotor sequences

40. Deficiency in vWF

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41. 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
Dendritic cells?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

42. Wilm's tumor - RCC - HCC - hydronephrosis
Hodgkin
Hereditary spherocytosis - G6PD - sickle cell
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
T(12;21)

43. Where are 1/3 of platelets stored
Orotic aciduria
Spleen
Inc vasodiltion - inc perm - inc pain
Fibrinogen

44. Spherocyte
Hereditary spherocytosis - autoimmune hemolysis
Pos
Reed - sternberg cells
Both alpha and beta thal

45. What are the labs and tx for HS?
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Uroporphyrin (tea colored urine)
Inc UCB
Positive osmootic fragility test and splenectomy

46. What is the treatment for acute intermittent porphyria
Anti Rh
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Prothrombin gene mutation
Glucose and heme - which inhibit ALA synthase

47. What substance is Fe added to to yield heme
MGUS - monoclonal gammopathy of undetermined significance
No lytic bone lesions in WM
Mantle cell - older males
Protoporphyrin

48. What is the mutation in HbC
Nl PT - elevated PTT - intrinsic pathway defect
Lysine for glutamate at position 6
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
IgG - Warm is GREAT

49. What is the affected enzyme in lead poisoning
Ferrocheletase and ALA dehydrogenase
Up - down - up
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Uroporphyrinogen decarboxylase

50. Ddx for microcytic anemia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Decrease EPO
Fe def - thal - pb poison - sideroblastic anemia