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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 are the presenting symptoms of porphyria cutanea tarda
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Blistering cutaneous photosens - most common porphyria
Inc UCB
EBV

2. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Schistocytes and inc LDH
Her next Rh+ fetus
Sideroblastic anemia
They bind vWF via GpIb

3. What is the pathogenesis of sickle cell
Up - down - up
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Varying sizes
No antigen - both antibodies

4. What is the defect in beta thal?
Splicing sites and promotor sequences
Bernard soulier
Inc HbF and dec HbS
Fibrinogen

5. Fava beans - sufla drugs - infectinos
Aspirin
Lysosomes
Examples of oxidative stress
Lead - and EtOH

6. What do you see a starry sky appearance in Burkitt
Sheets of lymphocytes interspersed with macs
Lysosomes
Bite cells and Heinz bodies
No

7. Adults present with cutaneous patches/nodules - indolent CD4+
Mycosis fundgoides/Sezary syndrome
90% anearobically from glucose to lactate - 10% from HMP shunt
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
1 to 6

8. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
Inc large vWF multimers - inc platelet aggregation and thrombosis
T(12;21)
DIC
Blood

9. bcl -2 t(14;18) adults
ADP and Ca
Follicular lymphoma - indolent course
Extravascular
Factor V Leiden

10. t(11:22)
Lung disease - congenital heart diseaes - and high altitude
Ewing sarcoma
Valine for glutamate
Schistocytes - helmet cells

11. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Sideroblastic anemia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Reed - sternberg cells

12. adults - auer rods - inc circulating myeblasts on peripheral smear
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Bimodal - men>women - except for nodular sclerosing type
AML
1 to 6

13. What is appropriate absolyte polycythemia associated with
Up - down - up
Lung disease - congenital heart diseaes - and high altitude
ATIII def
40 to 75

14. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Off center nuclues - clock face chromatin
Beta thal
40 to 75
Inc HbF and dec HbS

15. What role does antithrombin play?
B6 therapy (pyrodixine)
Sideroblastic anemia
Incactivates II - VII - IX - X - XI - XII
2 to 10

16. universal donor
Decrease EPO
Imatinib - anti bcr abl
No antigen - both antibodies
20 to 40

17. What percentage if WBCs are polys?
CD15 and CD30 pos
Hereditary spherocytosis
40 to 75
Sideroblastic anemia

18. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Spleen
Lung disease - congenital heart diseaes - and high altitude
Alpha thal - asian and african american
Sideroblastic anemia

19. What virus is associated with 50% of Hodgkin lymphoma
Exposed collagen upon endothelial damage
Inc lymphoctes - and less RS cells
MHC II and Fc receptors
EBV

20. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Langerhans cells
Epoxide reductase - warfarin inhibits
Bimodal - men>women - except for nodular sclerosing type
Def in factor IX

21. mother's antibodies attack fetal RBCs
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Alpha thal - asian and african american
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Inc HbF and dec HbS

22. Is G6PD intravascular or extravascular
Intravascular
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
MGUS - monoclonal gammopathy of undetermined significance
Bernard soulier

23. In hemophilia A or B What do you see in the coag tests
Low O2 in papilla; can also get microhematuria from medullary infarcts
Nl PT - elevated PTT - intrinsic pathway defect
Idiopathic thrombocytopenia (ITP)
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

24. universal recipient
AB - no antibodies
Bone marrow infiltration - myelofibrosis
Follicular lymphoma - indolent course
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

25. What are the azuraphilic granules in PMNs
Positive osmootic fragility test and splenectomy
Beta chain underproduced - asymptomatic - heterozygote
Lysosomes
Helminth infections major basic protein

26. How do platelet disorders present?
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
EBV
CML
ADP and Ca

27. Wilm's tumor - RCC - HCC - hydronephrosis
Bone marrow - thymus - blood (80% of circulating lymphos are T)
Abciximab
Howell Jolly bodies
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

28. What is the pathogenesis of aplastic anemia with kidney disease
Plasma cell
Low O2 in papilla; can also get microhematuria from medullary infarcts
Decrease EPO
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

29. Where are basophils found?
Blood
Inc HbA2 on electrophoresis
Uroporphyrinogen decarboxylase
Hereditary spherocytosis - autoimmune hemolysis

30. Ringed sideroblasts
Imatinib - anti bcr abl
Sideroblastic anemia
20 to 40
B antigena and A antibodes

31. How does the therapy for M3 vairant work?
Inc
Induces differentiation of myeloblasts
Up - down - nl
Low O2 in papilla; can also get microhematuria from medullary infarcts

32. What is the mutation in HbC
Lysine for glutamate at position 6
Sheets of lymphocytes interspersed with macs
VWF and fibrinogen
Lead - and EtOH

33. Plasma cell neoplasm
Multiple myeloma
Plasma cell
Dec plasma volume
Ferrocheletase and ALA dehydrogenase

34. What does plasmin do?
Protoporphyrin
Degrades fibrin mesh and converts C3 to C3a
VIII
Examples of oxidative stress

35. Back pain - hemoglobinuria
Mantle cell - older males
Anti Rh
Common a few days after oxidative stress in a pt with G6PD
Alpha thal - asian and african american

36. What is the Ddx for for a macrocytic anemia
Nl - up - nl
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
B6
Degrades fibrin mesh and converts C3 to C3a

37. CD5+ - poor prognosis - t(11;14)
Mantle cell - older males
Positive osmootic fragility test and splenectomy
Follicular lymphoma - indolent course
Abciximab

38. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Mantle cell - older males
Thrombotic thrombocytopenic purpura
40 to 75

39. What are some classic examples of extravascular hemolysis
IFN gama
Hereditary spherocytosis - G6PD - sickle cell
Liver disease - abetalipoproteinemia - acntho = spiny
Prothrombin gene mutation

40. What is appropriate absolute polycythemia
Fe def - thal - pb poison - sideroblastic anemia
Inc HbF and dec HbS
Inc RBC - dec O2 sat - inc EPO
Aplastic anemia - pancytopenia

41. What does antithrombin do and What activates it?
Down - up - down
Bone marrow infiltration - myelofibrosis
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Malaria - Babesia

42. Where do B cells arise from - mature - and migrate to...
VWF and fibrinogen
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Leukemia
VIII

43. What are the etiologies of folate def
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
IFN gama
M3 AML (acute promyelocytic leukemia)
All trans retinoic acid

44. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Spleen
They bind vWF via GpIb
Lacunar in nodular sclerosing variant
B12 def

45. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Langerhans cell histiocytosis
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 -
M3 AML (acute promyelocytic leukemia)
Hereditary spherocytosis

46. Which cell is neoplastic in multiple myeloma
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Plasma cell
MAHA
Mycosis fundgoides/Sezary syndrome

47. drug that inhibits ADP induced expression of GpIIb/IIIa
Ticlopidine/clopidogrel
Induces differentiation of myeloblasts
Exposed collagen upon endothelial damage
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

48. What does ADP do?
Exposed collagen upon endothelial damage
Helps platelts adhere to endothelium
Hairy cell leukemia
IgG - Warm is GREAT

49. deficiency in ADAMTS13 leading to dec degradation of vWF
Signs and sx of aplastic anemia
Polycythemia vera
Thrombotic thrombocytopenic purpura
All

50. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Lead - and EtOH
Inducers of primary antibody response
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning