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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 does the blood smear show in glanzmann's?
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Aplastic anemia - pancytopenia
No platelet clumping
DIC - TTP/HUS - traumatic hemolysis

2. What substances are released in mast cell degranulation
Polycythemia vera
Mycosis fundgoides/Sezary syndrome
Histamine - heparin - and eosinohil chemotactic factors
Adult T cell lymphoma - presents with cutaneous lesions

3. What are the age ranges for the various leukemias
CLL (SLL without the peripheral lymphocytosis
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Paraprotein spike - monoclonal protein
Histaminase and arylsulfatase

4. What is the tx for aplastic anemia
Lacunar in nodular sclerosing variant
EBV
Intravascular hemolysis
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

5. What does increasing heme do to ALA synthase activity
Follicular lymphoma - indolent course
Dec
Protein C or S deficiency
Leukemia

6. What is monoclonal expansion without symptoms associated with multiple myeloma?
Down - down - up
MGUS - monoclonal gammopathy of undetermined significance
Myelofibrosis (marow is crying being its fibrosed'
Inactivates it

7. What is the treatment for sideroblastic anemia
Beta 4
B6 therapy (pyrodixine)
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Fc

8. t(15;17)
Indirect coombs - agglutinate if serum anti RBC surface Ig
M3 AML (acute promyelocytic leukemia)
Hairy cell leukemia
Intrinsic - all factors except - VII - XIII

9. What do you see a starry sky appearance in Burkitt
Leukemoid reaction
Sheets of lymphocytes interspersed with macs
Produce antibody - lots of RER and golgi
Glycine and succinyl - coa

10. CD5+ - poor prognosis - t(11;14)
Porphyrias
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Mantle cell - older males
Birbeck granules

11. What are the etiologies of folate def
Mycosis fundgoides/Sezary syndrome
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Antigen - antibody
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

12. Schisotcyte - helmet cell
Antigen - antibody
Sickle cell anemia
Glycine and succinyl - coa
DIC - TTP/HUS - traumatic hemolysis

13. t(8;14) c - myc gene
Burkitt lymphoma
Intravascular hemolysis
Incactivates II - VII - IX - X - XI - XII
20 to 40

14. What happens in betal thal minor?
CML
Inc HbF and dec HbS
Beta chain underproduced - asymptomatic - heterozygote
SLE - CLL - alpha methyldopa

15. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
All
Mycosis fundgoides/Sezary syndrome
Anti Rh
T(12;21)

16. What do labs show in ITP?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
90% anearobically from glucose to lactate - 10% from HMP shunt
Aplastic anemia - pancytopenia
Alpha thal - asian and african american

17. What is the characteristic histologic finding in Hodgkin Lymphoma
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Paraprotein spike - monoclonal protein
Ewing sarcoma
Reed - sternberg cells

18. Conditions of defective heme synthesis leading to accumulation of heme precurors
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Porphyrias
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Blod - tissue - MACS

19. What does LEAD stand for in lead poisoning?
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
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 -
Fibrinogen bind GpIIb/IIIa and links platelts
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

20. What is the effected enzyme in acute intermittment porphyria?
Africa = Jaw lesion - US = pelvis or abdomen
Blod - tissue - MACS
Leukemoid reaction
Porphobilinogen deaminase aka uroporphyrinogen I synthase

21. What is a metabolic disorder tht can cause macrocytic anemia?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Orotic aciduria
Porphyrias
Hereditary spherocytosis - G6PD - sickle cell

22. Serum iron - transferrin - ferritin lab values for iron def anemia
Lacunar in nodular sclerosing variant
Dendritic cells?
Africa = Jaw lesion - US = pelvis or abdomen
Down - up - down

23. Where to T cells arise and mature - and migrate to...
B12 in fatty acid pathways leads to subacute combined degeneration
Bone marrow - thymus - blood (80% of circulating lymphos are T)
M3 AML (acute promyelocytic leukemia)
Glanzmann's throbmasthenia

24. Which substrates begin the heme synthesis pathway
Anemia of chronic disease - Aplastic anemia - kidney disease
PGI2 - NO inc blood flow - dec platelet aggregation
Glycine and succinyl - coa
IFN gama

25. Where are basophils found?
VWF and fibrinogen
Incactivates II - VII - IX - X - XI - XII
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Blood

26. S-100 and CD1a with birbeck granules
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Bleeding - malnutrition/absorption - inc demand pregs
Hereditary spherocytosis - G6PD - sickle cell
Langerhans cell histiocytosis

27. What begins the extrinsic pathway?
Leukemoid reaction
Tissue factor converst the VII and VIIa
Imatinib - anti bcr abl
Polycythemia vera

28. Who are the professional APCs?
Pos
Dendritic cells?
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
B antigena and A antibodes

29. Is G6PD intravascular or extravascular
Hairy cell leukemia
...
Intravascular
Helminth infections major basic protein

30. How does the therapy for M3 vairant work?
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Induces differentiation of myeloblasts
120 days
Paraprotein spike - monoclonal protein

31. Can B cells function as APCs?
Varying sizes
Inc HbF and dec HbS
Yes - via MHC II
Ewing sarcoma

32. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Alpha thal - asian and african american
Protein C or S deficiency
Inc HbF and dec HbS
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

33. inc HbF on electrophoresis
Both alpha and beta thal
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
All
Acts to stabilize platelet plug

34. anisocytosis
Varying sizes
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Lysosomes

35. In hemophilia A or B What do you see in the coag tests
Nl PT - elevated PTT - intrinsic pathway defect
No lytic bone lesions in WM
Dec
Malaria - Babesia

36. Does CML have a JAK2 mut
Kids= exposure to lead paint - adults = battery - ammunition factory
Birbeck granules
Positive osmootic fragility test and splenectomy
No

37. t(11:22)
Hairy cell leukemia
No platelet clumping
TXA2 - dec blood flow - inc platelet aggregation
Ewing sarcoma

38. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Uroporphyrin (tea colored urine)
They bind vWF via GpIb
SLE - CLL - alpha methyldopa
Ewing sarcoma

39. What is the presenting scenario for TTP?
Her next Rh+ fetus
AB - no antibodies
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Myelofibrosis (marow is crying being its fibrosed'

40. What do platelets interact with to form a hemostatic plug
Howell Jolly bodies
Contiguous
Fibrinogen
8-10 days

41. What does ADP do?
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Helps platelts adhere to endothelium
Induces differentiation of myeloblasts
...

42. What are the azuraphilic granules in PMNs
Sickle cell anemia
Collagen - BM - activated platelets
Lysosomes
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

43. What are the etiologies of B12 def
A antigen and B antibodies
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
G6PD
Hydroxyurea - bone marrow transplant

44. What is the characteristic lab finding on electrophoresis
Uroporphyrinogen decarboxylase
Incactivates II - VII - IX - X - XI - XII
Dec synthesis of factors 1972 - protein C/S
Paraprotein spike - monoclonal protein

45. What are some classic examples of extravascular hemolysis
Kids= exposure to lead paint - adults = battery - ammunition factory
Hereditary spherocytosis - G6PD - sickle cell
Intravascular hemolysis
90% anearobically from glucose to lactate - 10% from HMP shunt

46. Adults present with cutaneous patches/nodules - indolent CD4+
Mycosis fundgoides/Sezary syndrome
Epoxide reductase - warfarin inhibits
Bite cells and Heinz bodies
A antigen and B antibodies

47. What does Vit K deficiency cause?
Round densly staining nucleus with a small amount of pale cytoplasm
Dec synthesis of factors 1972 - protein C/S
Diffuse Large b cell lymphoma
Mantle cell - older males

48. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Nl - up - nl
IFN gama
Up - down - up
Parvovirus

49. What causes the jaundice in extravascular hemolysis
Macrohemorrhage - hemarthroses - easy bruising
Induces differentiation of myeloblasts
Up - down - up
Inc UCB

50. What is the affected enzyme in lead poisoning
Uroporphyrinogen decarboxylase
Ferrocheletase and ALA dehydrogenase
Degrades fibrin mesh and converts C3 to C3a
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia