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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. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
EBV
B6
Heinz bodies - seen in alpha thal and G6PD
Aspirin

2. What does ectopic EPO produce
Inappropriate absolute with inc RBCs and EPO
Multiple myeloma
Prothrombin gene mutation
Inc HbF and dec HbS

3. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Factor V resistant to activated protein C's inhibition
Paroxysmal nocturnal hemoglobinuria
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

4. Deficiency in GpIb
Bernard soulier
Inc large vWF multimers - inc platelet aggregation and thrombosis
Round densly staining nucleus with a small amount of pale cytoplasm
Mantle cell lymphoma

5. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
ATIII def
Off center nuclues - clock face chromatin
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
IgG - Warm is GREAT

6. What is the enzyme defect in Porphyria cutanea tarda
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Imatinib - anti bcr abl
Aplastic anemia - pancytopenia
Uroporphyrinogen decarboxylase

7. What is a blast crisis
CML to AML or All
B antigena and A antibodes
Inc large vWF multimers - inc platelet aggregation and thrombosis
Sideroblastic anemia

8. What are the four levels of alpha thal?
Low in CML
Blod - tissue - MACS
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Inappropriate absolute with inc RBCs and EPO

9. How are the nucleus and the cytoplasm characterized for lymphocytes
Liver disease - abetalipoproteinemia - acntho = spiny
Collagen - BM - activated platelets
Round densly staining nucleus with a small amount of pale cytoplasm
Her next Rh+ fetus

10. What virus is associated with 50% of Hodgkin lymphoma
Macrohemorrhage - hemarthroses - easy bruising
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Rhogam - Rh antigen immunoglobulin
EBV

11. What is the Ddx for for a macrocytic anemia
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
VWF carries/protects factor VIII
G6PD
Protoporphyrin

12. What do plasma cells do
Uroporphyrinogen decarboxylase
Hereditary spherocytosis
Epoxide reductase - warfarin inhibits
Produce antibody - lots of RER and golgi

13. What is the affected enzyme in acute intermittment porphyria
Porphobilinogen - delta ALA - uroporphyrin
Fe def - thal - pb poison - sideroblastic anemia
B symptoms - fever night sweats - weight loss
Alpha thal - asian and african american

14. anisocytosis
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
...
5- FU - AZT - hydroxyurea
Varying sizes

15. What percentage if WBCs are polys?
Easy gas exchange
Yes - via MHC II
Ringed sideronblasts with iron laden mitochondria
40 to 75

16. What is the age group most commonly affected by multiple myeloma
Spleen
AML
40 to 50
VWD

17. How does vWD cause elevated PTT?
IgG - Warm is GREAT
Acts to stabilize platelet plug
CML to AML or All
VWF carries/protects factor VIII

18. poikilocytosis
Varying shapes
Sheets of lymphocytes interspersed with macs
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Inducers of primary antibody response

19. What is the effect of ACE on bradykinin
Alpha thal - asian and african american
Bimodal - men>women - except for nodular sclerosing type
Dec plasma volume
Inactivates it

20. How does the therapy for M3 vairant work?
Decrease EPO
Langerhans cells
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Induces differentiation of myeloblasts

21. Ringed sideroblasts
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Hereditary spherocytosis - autoimmune hemolysis
2 to 10
Sideroblastic anemia

22. tennis rackets on EM
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Splicing sites and promotor sequences
IgG - Warm is GREAT
Birbeck granules

23. Causes of iron def?
Glycine and succinyl - coa
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Epoxide reductase - warfarin inhibits
Bleeding - malnutrition/absorption - inc demand pregs

24. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Follicular lymphoma - indolent course
Varying shapes
They bind vWF via GpIb

25. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Contiguous
Hereditary spherocytosis
Fe def - thal - pb poison - sideroblastic anemia
Glycine and succinyl - coa

26. What is the coombs test results in AIHA?
Spleen
SLE - CLL - alpha methyldopa
Tissue factor converst the VII and VIIa
Pos

27. Upregulated growth of leukocytes in bone marro
Leukemia
Malaria - Babesia
Ferrocheletase and ALA dehydrogenase
Degrades fibrin mesh and converts C3 to C3a

28. What virus can cause an aplastic crisis in pts with HS?
Parvovirus
1 to 6
Heinz bodies - seen in alpha thal and G6PD
PGI2 - NO inc blood flow - dec platelet aggregation

29. What does increasing heme do to ALA synthase activity
Dec
Alpha thal - asian and african american
Macrohemorrhage - hemarthroses - easy bruising
Low in CML

30. Adults present with cutaneous patches/nodules - indolent CD4+
Glucose and heme - which inhibit ALA synthase
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Mycosis fundgoides/Sezary syndrome
Extravascular hemolysis

31. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Protein C or S deficiency
Thrombotic thrombocytopenic purpura
PGI2 - NO inc blood flow - dec platelet aggregation
Def in factor IX

32. Where are mast cells found
Malaria - Babesia
CLL (SLL without the peripheral lymphocytosis
VWD
Tissue

33. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Beta thal
Blistering cutaneous photosens - most common porphyria
Burkitt lymphoma
CLL (SLL without the peripheral lymphocytosis

34. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Nl - up - nl
Low in CML
Hb Barts - gamma4 in defect in all 4 alpha genes
Produce antibody - lots of RER and golgi

35. Megakaryocytosis
Low O2 in papilla; can also get microhematuria from medullary infarcts
Follicular lymphoma - indolent course
Essential thrombocytosis
B symptoms - fever night sweats - weight loss

36. What does the blood smear show in glanzmann's?
Acts to stabilize platelet plug
No platelet clumping
Nodular sclerosing
Positive osmootic fragility test and splenectomy

37. Teardrop cell
Intravascular hemolysis
Howell Jolly bodies
Bone marrow infiltration - myelofibrosis
Inc large vWF multimers - inc platelet aggregation and thrombosis

38. What does ADP do?
Aspirin
Varying sizes
Helps platelts adhere to endothelium
DDAVP (desmopressin) which releases stored vWF stored in endothelium

39. What do eosinophils defend against and What do they use to do it?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Helminth infections major basic protein
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Multiple myeloma

40. What is their role?
Inducers of primary antibody response
Schistocytes - helmet cells
MHC II and Fc receptors
Fc

41. What is the characteristic histologic finding in Hodgkin Lymphoma
...
GpIIb/IIIa
Reed - sternberg cells
Alpha thal - asian and african american

42. t(8;14) c - myc gene
Burkitt lymphoma
Fe def - thal - pb poison - sideroblastic anemia
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
VWF and fibrinogen

43. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Off center nuclues - clock face chromatin
Her next Rh+ fetus
Glanzmann's throbmasthenia
Mantle cell - older males

44. What does plasmin do?
Degrades fibrin mesh and converts C3 to C3a
Fe def - thal - pb poison - sideroblastic anemia
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
G6PD

45. What is the pathogenesis of sickle cell
Uroporphyrinogen decarboxylase
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Cromolyn sodium
CML to AML or All

46. Basophilic nuclear remnants fonud in RBCs
Positive osmootic fragility test and splenectomy
Howell - Jolly bodies - hypo/asplenia
No
Thrombotic thrombocytopenic purpura

47. Eosinophils are highly phagocytic For what kind of complex?
Ewing sarcoma
Africa = Jaw lesion - US = pelvis or abdomen
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Antigen - antibody

48. What do labs show in DIC?
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
DIC - TTP/HUS - traumatic hemolysis
Beta thal

49. What are the variants of the RS cells
Lacunar in nodular sclerosing variant
Hereditary elloptocytosis
Macrohemorrhage - hemarthroses - easy bruising
Blood

50. What do you see a starry sky appearance in Burkitt
Sheets of lymphocytes interspersed with macs
Nodular sclerosing
Mantle cell lymphoma
40 to 50