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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 are some causes of warm agglutinin autoimmune hemolytic anemia?
Sideroblastic anemia
Diffuse Large b cell lymphoma
B6
SLE - CLL - alpha methyldopa

2. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
Lacunar in nodular sclerosing variant
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
No lytic bone lesions in WM
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

3. 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
20 to 40
Epoxide reductase - warfarin inhibits
Sickle cell
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

4. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Paraprotein spike - monoclonal protein
Glanzmann's throbmasthenia
Alpha thal - asian and african american
Sickle cell anemia

5. What do you see in peripheral smear in a pt with G6PD?
Tissue factor converst the VII and VIIa
Antigen - antibody
Bite cells and Heinz bodies
Hereditary elloptocytosis

6. Where are mast cells found
Sheets of lymphocytes interspersed with macs
Lacunar in nodular sclerosing variant
Tissue
VWF and fibrinogen

7. universal donor
120 days
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Cromolyn sodium
No antigen - both antibodies

8. What is a blast crisis
CML to AML or All
Examples of oxidative stress
5- FU - AZT - hydroxyurea
Reed - sternberg cells

9. What is the pattern of involvement and spread for nonHod lympho
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Epoxide reductase - warfarin inhibits
Howell - Jolly bodies - hypo/asplenia

10. What does plasmin do?
Low O2 in papilla; can also get microhematuria from medullary infarcts
Hb Barts - gamma4 in defect in all 4 alpha genes
Inc vasodiltion - inc perm - inc pain
Degrades fibrin mesh and converts C3 to C3a

11. What is the tx for vWD
Blod - tissue - MACS
Deficiency in factor VIII
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Contiguous

12. What is appropriate absolute polycythemia
Fc
Uroporphyrin (tea colored urine)
Inc RBC - dec O2 sat - inc EPO
Africa = Jaw lesion - US = pelvis or abdomen

13. What is makes a leukemia acute
Up - down - nl
Inactivates it
Sideroblastic anemia
Blasts > 5%

14. Schisotcyte - helmet cell
DIC - TTP/HUS - traumatic hemolysis
Lysine for glutamate at position 6
Nl PT - elevated PTT - intrinsic pathway defect
No

15. When do you see MAHA?
Gp1b
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
CML to AML or All

16. What does LEAD stand for in lead poisoning?
Malaria - Babesia
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 -
A antigen and B antibodies
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

17. Fava beans - sufla drugs - infectinos
Protoporphyrin (blood)
Examples of oxidative stress
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
B6 therapy (pyrodixine)

18. What is hemophilia B?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Follicular lymphoma - indolent course
Def in factor IX
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

19. anisocytosis
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Varying sizes
CLL (SLL without the peripheral lymphocytosis
IFN gama

20. What does bradykinin do?
Ticlopidine/clopidogrel
Inappropriate absolute with inc RBCs and EPO
Inc vasodiltion - inc perm - inc pain
Hereditary spherocytosis - autoimmune hemolysis

21. t(11;14)
Mantle cell lymphoma
G6PD
Up - down - nl
Howell - Jolly bodies - hypo/asplenia

22. What does the blood smear show in glanzmann's?
No platelet clumping
Down - up - down
Low O2 in papilla; can also get microhematuria from medullary infarcts
TXA2 - dec blood flow - inc platelet aggregation

23. Upregulated growth of leukocytes in bone marro
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Produce antibody - lots of RER and golgi
Contiguous
Leukemia

24. fatigue - malaise - pallor - purpura - mucosal bleeding - petechiae - infection
40 to 50
Signs and sx of aplastic anemia
Birbeck granules
All trans retinoic acid

25. Basophilic nuclear remnants fonud in RBCs
Howell - Jolly bodies - hypo/asplenia
Peroxidase
Glucose and heme - which inhibit ALA synthase
Antigen - antibody

26. t(15;17)
M3 AML (acute promyelocytic leukemia)
Megakaryocytes
B6 therapy (pyrodixine)
Nodular sclerosing

27. Is G6PD intravascular or extravascular
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Low in CML
Plasma cell
Intravascular

28. Which factor does vWF carry/protect
VIII
Hairy cell leukemia
T(9;22) bcr abl
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

29. What is the effect of ACE on bradykinin
ADP and Ca
EBV
Multiple myeloma
Inactivates it

30. What is the purpose of the fibrin mesh
Examples of oxidative stress
Acts to stabilize platelet plug
IFN gama
No lytic bone lesions in WM

31. What does CRAB stand for in multiple myeloma
Hydroxyurea - bone marrow transplant
Hairy cell leukemia
Paroxysmal nocturnal hemoglobinuria
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain

32. How is beta thal minor dx
Inc HbA2 on electrophoresis
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
M3 AML (acute promyelocytic leukemia)
Leukemoid reaction

33. What do the iron studies show in sideroblastic anemia
Essential thrombocytosis
Inc serum iron - normal TIBC - inc ferratin
Inactivates it
Burkitt lymphoma

34. universal recipient
Hereditary spherocytosis
G6PD
Bimodal - men>women - except for nodular sclerosing type
AB - no antibodies

35. Which cell is neoplastic in multiple myeloma
Factor V Leiden
Paraprotein spike - monoclonal protein
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Plasma cell

36. Drug that inhbits the GpIIb/IIIa directly
Abciximab
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Easy gas exchange
B antigena and A antibodes

37. What is their role?
Both alpha and beta thal
B symptoms - fever night sweats - weight loss
Off center nuclues - clock face chromatin
Inducers of primary antibody response

38. What is the are the presenting symptoms of lead poisoning in kids and adults
Lacunar in nodular sclerosing variant
CD15 and CD30 pos
Deficiency in factor VIII
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

39. What reveresible things can a sideroblastic anemia
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Extravascular
Paroxysmal nocturnal hemoglobinuria
Lead - and EtOH

40. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Factor V Leiden
VWF and fibrinogen
ADP and Ca
Macrohemorrhage - hemarthroses - easy bruising

41. philadelphia chromosome - blood looks like marrow
CML
Sickle cell anemia
Howell Jolly bodies
VWF and fibrinogen

42. What does hairy cell leukemia stain with
DIC
Burkitt lymphoma
Abciximab
TRAP (tartrate resistant acid phosphatase

43. What is the receptor for fibrinogen?
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Inappropriate absolute with inc RBCs and EPO
GpIIb/IIIa

44. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Acts to stabilize platelet plug
Blod - tissue - MACS
Mycosis fundgoides/Sezary syndrome
Complications of sickle cell anemia

45. What is the most common nonhodgkin lymphoma
Hairy cell leukemia
Sickle cell
Diffuse Large b cell lymphoma
Glycine and succinyl - coa

46. What does the large SA:volume ratio in RBCs help facilitate?
Myelofibrosis (marow is crying being its fibrosed'
Glycine and succinyl - coa
Down - down - up
Easy gas exchange

47. What chromosomal translocation is associated with a better prognosis in All
GpIIb/IIIa
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
T(12;21)
DDAVP (desmopressin) which releases stored vWF stored in endothelium

48. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Up - down - nl
Alpha thal - asian and african american
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Blistering cutaneous photosens - most common porphyria

49. Where do you see hypersegmented polys?
B antigena and A antibodes
Megakaryocytes
Tissue
B12/folate def

50. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Von Willebrand's disease
IgG - Warm is GREAT