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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. Elderly - mature b cell tumor with filamentous - hairlike projections
Alpha thal - asian and african american
Hairy cell leukemia
8-10 days
DIC - TTP/HUS - traumatic hemolysis

2. discrete tumor masses arising from lymph nodes
Beta 4
Plasma cell
Lymphoma
Aspirin

3. tennis rackets on EM
Birbeck granules
Glanzmann's throbmasthenia
Down - up - down
All

4. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Anti Rh
TRAP (tartrate resistant acid phosphatase
HIV or immunosupression
Factor V Leiden

5. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Down - down - up
CD15 and CD30 pos
Protein C or S deficiency
Dec synthesis of factors 1972 - protein C/S

6. What are the main associations with multiple myeloma?
Inc RBC - dec O2 sat - inc EPO
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Histaminase and arylsulfatase
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

7. philadelphia chromosome - blood looks like marrow
B12 def
Polycythemia vera
CML
Beta thal

8. What are the etiologies of folate def
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
T(9;22) bcr abl
Degrades fibrin mesh and converts C3 to C3a
Ferrocheletase and ALA dehydrogenase

9. What is the receptor for vWF
2 to 10
Dec synthesis of factors 1972 - protein C/S
Gp1b
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

10. What does the blood smear show in glanzmann's?
No platelet clumping
Deficiency in factor VIII
Off center nuclues - clock face chromatin
Protein C or S deficiency

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

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12. What are the extrinsic hemolytic normocytic anemias?
Nl - up - nl
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 -
...
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

13. What virus can cause an aplastic crisis in pts with HS?
Parvovirus
Inc HbA2 on electrophoresis
Sickle cell
Peroxidase

14. What is the tx for sickle cell
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Down - up - down
Hydroxyurea - bone marrow transplant

15. What is appropriate absolute polycythemia
2 to 10
Inc RBC - dec O2 sat - inc EPO
Malaria - Babesia
Beta chain underproduced - asymptomatic - heterozygote

16. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Extravascular hemolysis
Idiopathic thrombocytopenia (ITP)
Exposed collagen upon endothelial damage
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

17. What is the treatment for acute intermittent porphyria
IgG - Warm is GREAT
Leukemia
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Glucose and heme - which inhibit ALA synthase

18. What does antithrombin do and What activates it?
90% anearobically from glucose to lactate - 10% from HMP shunt
Thrombotic thrombocytopenic purpura
Lacunar in nodular sclerosing variant
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

19. What virus is associated with 50% of Hodgkin lymphoma
Hairy cell leukemia
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Inc UCB
EBV

20. What percentage if WBCs are polys?
VWF and fibrinogen
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Porphyrias
40 to 75

21. Spherocyte
Hereditary spherocytosis - autoimmune hemolysis
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Inc large vWF multimers - inc platelet aggregation and thrombosis

22. What is the general pathology of a macrocytic anemia?
Aplastic anemia - pancytopenia
TPA - cleavage of fibrin mesh
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Glycine and succinyl - coa

23. Fibrotic obliteration of bone marow with teardrop cells

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24. What virus is associated with Burkitt lymphoma
Essential thrombocytosis
Diffuse Large b cell lymphoma
Paraprotein spike - monoclonal protein
EBV

25. What is the effect of ACE on bradykinin
All
CLL (SLL without the peripheral lymphocytosis
Incactivates II - VII - IX - X - XI - XII
Inactivates it

26. Megakaryocytosis
Essential thrombocytosis
Contiguous
SLE - CLL - alpha methyldopa
Spleen

27. What is the Ddx for nonhemolytic normocytic anemia
Anemia of chronic disease - Aplastic anemia - kidney disease
Extrinsic - I - II - V - VII and X
CNS and testis
MGUS - monoclonal gammopathy of undetermined significance

28. Eosinophils are highly phagocytic For what kind of complex?
Antigen - antibody
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Leukemoid reaction
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

29. What substance accumulates in porphyria cutanea
Uroporphyrin (tea colored urine)
Alpha thal - asian and african american
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

30. What are the anti aggregation factors?
Induces differentiation of myeloblasts
PGI2 - NO inc blood flow - dec platelet aggregation
No platelet clumping
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

31. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Bite cells and Heinz bodies
No lytic bone lesions in WM
VIII
Sideroblastic anemia

32. What is the effected enzyme in acute intermittment porphyria?
Porphobilinogen deaminase aka uroporphyrinogen I synthase
CML
No lytic bone lesions in WM
Sickle cell

33. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Nl PT - elevated PTT - intrinsic pathway defect
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Polycythemia vera
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

34. In hemophilia A or B What do you see in the coag tests
Gp1b
Nl PT - elevated PTT - intrinsic pathway defect
Birbeck granules
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

35. Acanthocyte (spur cell)
Liver disease - abetalipoproteinemia - acntho = spiny
Anti Rh
Hairy cell leukemia
Peroxidase

36. What is the pathogenesis of sickle cell
Mycosis fundgoides/Sezary syndrome
Ticlopidine/clopidogrel
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Beta thal

37. 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
Anti Rh
Both alpha and beta thal

38. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Up - down - nl
Ankryin - band 4.1 or spectrin
VIII
TRAP (tartrate resistant acid phosphatase

39. Which factor does vWF carry/protect
Bite cells and Heinz bodies
Aspirin
CNS and testis
VIII

40. What is the difference of presentation of Burkitt in Africa vs the United States
Africa = Jaw lesion - US = pelvis or abdomen
Reed - sternberg cells
Mantle cell - older males
Ferrocheletase and ALA dehydrogenase

41. What is the treatment for sideroblastic anemia
Nl - up - nl
Mantle cell lymphoma
CML
B6 therapy (pyrodixine)

42. What activates the fibrinolytic pathway?
Beta chain underproduced - asymptomatic - heterozygote
Inappropriate absolute with inc RBCs and EPO
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Inc vasodiltion - inc perm - inc pain

43. What do labs show in ITP?
Helminth infections major basic protein
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Burkitt lymphoma
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

44. Does CML have a JAK2 mut
No
Dec
Down - up - down
EBV

45. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
Eleveated PT - PTT
Beta thal
Follicular lymphoma - indolent course
Nl - up - nl

46. What is the therapy for the M3 variant?
Factor V resistant to activated protein C's inhibition
Splicing sites and promotor sequences
All trans retinoic acid
Inc serum iron - normal TIBC - inc ferratin

47. What does bradykinin do?
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Adult T cell lymphoma - presents with cutaneous lesions
Inc vasodiltion - inc perm - inc pain
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

48. When do you see MAHA?
DIC
Fibrinogen bind GpIIb/IIIa and links platelts
Direct coombs - agglutinate if RBCs are coated with Ig
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis

49. What portion of IgE can mast cells bind
Schistocytes - helmet cells
Fc
...
Beta chain underproduced - asymptomatic - heterozygote

50. Deficiency in vWF

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