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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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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. What is hemophiliia A
Howell - Jolly bodies - hypo/asplenia
Deficiency in factor VIII
Factor V Leiden
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

2. What is the enzyme defect in Porphyria cutanea tarda
Easy gas exchange
Uroporphyrinogen decarboxylase
Howell - Jolly bodies - hypo/asplenia
Reed - sternberg cells

3. Causes of iron def?
Bleeding - malnutrition/absorption - inc demand pregs
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Beta 4
Dec plasma volume

4. Deficiency in GpIb
Petechiae
Bernard soulier
Porphyrias
Blod - tissue - MACS

5. What does plasmin do?
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Complications of sickle cell anemia
Degrades fibrin mesh and converts C3 to C3a
Inc HbF and dec HbS

6. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Macrohemorrhage - hemarthroses - easy bruising
Decrease EPO
Follicular lymphoma - indolent course
SLE - CLL - alpha methyldopa

7. drug that inhibits ADP induced expression of GpIIb/IIIa
Hereditary elloptocytosis
Adult T cell lymphoma - presents with cutaneous lesions
Ticlopidine/clopidogrel
Blood

8. Is G6PD intravascular or extravascular
B12 in fatty acid pathways leads to subacute combined degeneration
Intravascular
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Porphobilinogen deaminase aka uroporphyrinogen I synthase

9. Adults present with cutaneous patches/nodules - indolent CD4+
Hydroxyurea - bone marrow transplant
Mycosis fundgoides/Sezary syndrome
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

10. Which pathway and factors are tested in the PTT test
Sideroblastic anemia
Leukemia
Von Willebrand's disease
Intrinsic - all factors except - VII - XIII

11. What is relative polycythemia
No platelet clumping
Pyruvate kinase def - extravascular
Dec plasma volume
Hereditary spherocytosis - G6PD - sickle cell

12. Defect in platelet to platelet adhesion - receptor - disorder - platelet count and bleeding time

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13. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
CLL (SLL without the peripheral lymphocytosis
MGUS - monoclonal gammopathy of undetermined significance
GpIIb/IIIa
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

14. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Petechiae
Paroxysmal nocturnal hemoglobinuria
Orotic aciduria
Sideroblastic anemia

15. What is the tx for aplastic anemia
Incactivates II - VII - IX - X - XI - XII
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Complications of sickle cell anemia
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

16. What activates protein C and What does activated protein C do?
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Protein S - cleaves and invactivates Va and VIIIa
2 to 10

17. t(15;17)
Easy gas exchange
Induces differentiation of myeloblasts
M3 AML (acute promyelocytic leukemia)
Imatinib - anti bcr abl

18. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Low in CML
Schistocytes - helmet cells
Epoxide reductase - warfarin inhibits
Spleen

19. What are the presenting symptoms of acute intermittent porphyria
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Common a few days after oxidative stress in a pt with G6PD
PGI2 - NO inc blood flow - dec platelet aggregation
Mantle cell lymphoma

20. What do they express on their surface?
MHC II and Fc receptors
Inc serum iron - normal TIBC - inc ferratin
Leukemoid reaction
Multiple myeloma

21. What is a blast crisis
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Dec plasma volume
CML to AML or All
DIC

22. What is the therapy for the M3 variant?
Macrohemorrhage - hemarthroses - easy bruising
Splicing sites and promotor sequences
All trans retinoic acid
DIC - TTP/HUS - traumatic hemolysis

23. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
20 to 40
B12 def
DIC
Beta 4

24. What do the dense granules contain in platelets
Antigen - antibody
ADP and Ca
Fc
Kids= exposure to lead paint - adults = battery - ammunition factory

25. What are the presenting symptoms of porphyria cutanea tarda
Leukemia
No
Blistering cutaneous photosens - most common porphyria
2 to 10

26. Elderly - mature b cell tumor with filamentous - hairlike projections
TRAP (tartrate resistant acid phosphatase
Hairy cell leukemia
Sickle cell
CML to AML or All

27. iron deficiency anemia - esophageal web - atrophic glossitis
Epoxide reductase - warfarin inhibits
Plummer - vinson syndrome
Inc lymphoctes - and less RS cells
Nl - up - nl

28. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
TPA - cleavage of fibrin mesh
Leukemia
Rhogam - Rh antigen immunoglobulin
Both alpha and beta thal

29. What reveresible things can a sideroblastic anemia
Glycine and succinyl - coa
Lead - and EtOH
Collagen - BM - activated platelets
Ewing sarcoma

30. t(11:22)
Ewing sarcoma
Valine for glutamate
Hairy cell leukemia
Produce antibody - lots of RER and golgi

31. What activates the intrinsic pathway?
Lacunar in nodular sclerosing variant
No lytic bone lesions in WM
Collagen - BM - activated platelets
Anemia of chronic disease - Aplastic anemia - kidney disease

32. Sickle cell
Hairy cell leukemia
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Histaminase and arylsulfatase
Sickle cell anemia

33. What begins the extrinsic pathway?
Aplastic anemia - pancytopenia
40 to 50
Antigen - antibody
Tissue factor converst the VII and VIIa

34. How do platelet disorders present?
MAHA
Incactivates II - VII - IX - X - XI - XII
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface

35. What is the effect of ACE on bradykinin
Aspirin
Inactivates it
Sheets of lymphocytes interspersed with macs
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

36. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
Multiple myeloma
Paraprotein spike - monoclonal protein
ATIII def
Protoporphyrin

37. Mutation in 3' untranslated region associated with venous clots
Incactivates II - VII - IX - X - XI - XII
Inducers of primary antibody response
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Prothrombin gene mutation

38. What do you see on peripheral smear with sideroblastic anemia
Extrinsic - I - II - V - VII and X
Ringed sideronblasts with iron laden mitochondria
MHC II and Fc receptors
Down - up - down

39. Who has more severe disease - HbSS or HbSC
DDAVP (desmopressin) which releases stored vWF stored in endothelium
HbSS
PGI2 - NO inc blood flow - dec platelet aggregation
DIC

40. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Hereditary spherocytosis - G6PD - sickle cell
Beta thal
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

41. Which pathway and factorrs are tested by the PT coag test
SLE - CLL - alpha methyldopa
Anti Rh
Von Willebrand's disease
Extrinsic - I - II - V - VII and X

42. Drug that inhibits COX and therefore TXA2 synthesis
EBV
Aspirin
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Bleeding - malnutrition/absorption - inc demand pregs

43. Fava beans - sufla drugs - infectinos
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
5- FU - AZT - hydroxyurea
Liver disease - abetalipoproteinemia - acntho = spiny
Examples of oxidative stress

44. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Down - down - up
Degrades fibrin mesh and converts C3 to C3a
Anemia of chronic disease - Aplastic anemia - kidney disease
Glycine and succinyl - coa

45. Which cell is neoplastic in multiple myeloma
...
Def in factor IX
Gp1b
Plasma cell

46. What causes hydrops fetalis
Nl PT - elevated PTT - intrinsic pathway defect
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Hb Barts - gamma4 in defect in all 4 alpha genes
Inc large vWF multimers - inc platelet aggregation and thrombosis

47. Deficiency in vWF

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48. What virus is associated with Burkitt lymphoma
Heinz bodies - seen in alpha thal and G6PD
Pos
TXA2 - dec blood flow - inc platelet aggregation
EBV

49. Which maternal antibodies cross the placenta - anti A/B or anti Rh
120 days
Inc serum iron - normal TIBC - inc ferratin
B6
Anti Rh

50. What does bradykinin do?
Howell - Jolly bodies - hypo/asplenia
Megakaryocytes
Inc vasodiltion - inc perm - inc pain
Anti Rh