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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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This is a study tool. The 3 wrong answers for each question are randomly chosen from answers to other questions. So, you might find at times the answers obvious, but you will see it re-enforces your understanding as you take the test each time.

1. Where do B cells arise from - mature - and migrate to...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Thrombotic thrombocytopenic purpura
GpIIb/IIIa
Up - down - up

2. What virus can cause an aplastic crisis in pts with HS?
Inc serum iron - normal TIBC - inc ferratin
Fe def - thal - pb poison - sideroblastic anemia
Def in factor IX
Parvovirus

3. Which pathway and factorrs are tested by the PT coag test
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Extrinsic - I - II - V - VII and X
Tissue
B antigena and A antibodes

4. What is the treatment for acute intermittent porphyria
Extravascular
EBV
Glucose and heme - which inhibit ALA synthase
Leukemia

5. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Langerhans cells
Aplastic anemia - pancytopenia
Down - down - up
Leukemia

6. In hemophilia A or B What do you see in the coag tests
G6PD
Mantle cell - older males
Nl PT - elevated PTT - intrinsic pathway defect
Lysine for glutamate at position 6

7. What is the mutation in HbS
Bone marrow infiltration - myelofibrosis
Valine for glutamate
Multiple myeloma
Macrohemorrhage - hemarthroses - easy bruising

8. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Sideroblastic anemia
Porphobilinogen - delta ALA - uroporphyrin
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Ringed sideronblasts with iron laden mitochondria

9. Elderly - mature b cell tumor with filamentous - hairlike projections
Hairy cell leukemia
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
No platelet clumping
EBV

10. How do platelet disorders present?
Porphobilinogen - delta ALA - uroporphyrin
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Sideroblastic anemia
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

11. What do the iron studies show in sideroblastic anemia
Inc serum iron - normal TIBC - inc ferratin
Intrinsic - all factors except - VII - XIII
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

12. What is HbH
Beta 4
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Sickle cell anemia
Imatinib - anti bcr abl

13. What is the tx for aplastic anemia
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Hereditary spherocytosis
Tissue
Round densly staining nucleus with a small amount of pale cytoplasm

14. Which cell is neoplastic in multiple myeloma
Alpha thal - asian and african american
Plasma cell
Up - down - nl
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

15. Serum iron - transferrin - ferritin lab values for iron def anemia
Hereditary spherocytosis - autoimmune hemolysis
Down - up - down
Lead - and EtOH
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

16. What signal activates MACS
IFN gama
Degrades fibrin mesh and converts C3 to C3a
Her next Rh+ fetus
Glucose and heme - which inhibit ALA synthase

17. Why can newborns with sickle cell be asymptomatic
Off center nuclues - clock face chromatin
Inc HbF and dec HbS
Heinz bodies - seen in alpha thal and G6PD
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

18. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
Signs and sx of aplastic anemia
Indirect coombs - agglutinate if serum anti RBC surface Ig
Idiopathic thrombocytopenia (ITP)
CML

19. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
CLL (SLL without the peripheral lymphocytosis
Beta thal
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Intravascular hemolysis

20. What is the receptor for fibrinogen?
...
GpIIb/IIIa
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

21. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Idiopathic thrombocytopenia (ITP)
Sickle cell
T(9;22) bcr abl

22. Causes of iron def?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Bleeding - malnutrition/absorption - inc demand pregs
Dendritic cells?
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

23. What is the pathogenesis of aplastic anemia with kidney disease
Mantle cell - older males
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Rhogam - Rh antigen immunoglobulin
Decrease EPO

24. What is the ddx for aplastic anemia

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25. What is the characteristic lab finding on electrophoresis
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Paraprotein spike - monoclonal protein
Easy gas exchange

26. How are plasma cells characterized?
Off center nuclues - clock face chromatin
Orotic aciduria
Indirect coombs - agglutinate if serum anti RBC surface Ig
Factor V Leiden

27. Where are 1/3 of platelets stored
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Varying shapes
Sheets of lymphocytes interspersed with macs
Spleen

28. What is the pathogenesis of sickle cell
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Histamine - heparin - and eosinohil chemotactic factors
No
Fc

29. Where to T cells arise and mature - and migrate to...
Bone marrow - thymus - blood (80% of circulating lymphos are T)
B12 in fatty acid pathways leads to subacute combined degeneration
Paraprotein spike - monoclonal protein
Mantle cell - older males

30. What causes hydrops fetalis
Extrinsic - I - II - V - VII and X
A antigen and B antibodies
Low O2 in papilla; can also get microhematuria from medullary infarcts
Hb Barts - gamma4 in defect in all 4 alpha genes

31. What converts plasminogen to plasm and What does plasmin do?
SLE - CLL - alpha methyldopa
TPA - cleavage of fibrin mesh
Prothrombin gene mutation
Erythroblastosis fetalis - Rh or other blood antigen incompatibility

32. Schisotcyte - helmet cell
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Direct coombs - agglutinate if RBCs are coated with Ig
DIC - TTP/HUS - traumatic hemolysis
Factor V Leiden

33. What does hairy cell leukemia stain with
Down - down - up
Beta thal
TRAP (tartrate resistant acid phosphatase
Intrinsic - all factors except - VII - XIII

34. t(11:22)
Ewing sarcoma
Protein C or S deficiency
Yes - via MHC II
Howell Jolly bodies

35. Wilm's tumor - RCC - HCC - hydronephrosis
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Petechiae
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Hairy cell leukemia

36. What does decreasing heme do to ALA synthase activity?
Extrinsic - I - II - V - VII and X
B cells
Inc
DIC

37. What does Vit K deficiency cause?
Dec synthesis of factors 1972 - protein C/S
VWD
Blistering cutaneous photosens - most common porphyria
Up - down - nl

38. Deficiency in GpIb
Inc UCB
B antigena and A antibodes
Fc
Bernard soulier

39. What are the azuraphilic granules in PMNs
Von Willebrand's disease
Up - down - nl
Lysosomes
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

40. Deficiency in GpIIb/IIIa

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41. Fibrotic obliteration of bone marow with teardrop cells

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42. What do eosinophils defend against and What do they use to do it?
Low in CML
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Intrinsic - all factors except - VII - XIII
Helminth infections major basic protein

43. What is hemophilia B?
Both alpha and beta thal
Def in factor IX
Inc
Induces differentiation of myeloblasts

44. What is the most common nonhodgkin lymphoma
Nodular sclerosing
Africa = Jaw lesion - US = pelvis or abdomen
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Diffuse Large b cell lymphoma

45. What is the Ddx for nonhemolytic normocytic anemia
Lysine for glutamate at position 6
IgG - Warm is GREAT
Anemia of chronic disease - Aplastic anemia - kidney disease
Incactivates II - VII - IX - X - XI - XII

46. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Intravascular
120 days
Up - down - nl
Hereditary elloptocytosis

47. What do labs show in DIC?
Indirect coombs - agglutinate if serum anti RBC surface Ig
Low O2 in papilla; can also get microhematuria from medullary infarcts
HIV or immunosupression
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

48. What chromosomal translocation is associated with a better prognosis in All
MAHA
Intravascular
Histamine - heparin - and eosinohil chemotactic factors
T(12;21)

49. Which immunoglobulin is involved in warm agglutination?
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
IgG - Warm is GREAT
Birbeck granules
Dendritic cells?

50. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Hodgkin
EBV
AML
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin