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Hemeonc

Instructions:

Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
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1. Back pain - hemoglobinuria
Protoporphyrin
Common a few days after oxidative stress in a pt with G6PD
Collagen - BM - activated platelets
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia

2. What are the azuraphilic granules in PMNs
Lysosomes
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
40 to 75
Porphyrias

3. What do the platelets bind? What is the step called
Rhogam - Rh antigen immunoglobulin
They bind vWF via GpIb
CNS and testis
Off center nuclues - clock face chromatin

4. Which lymphoma is characterized by localized involvement with a single group of nodes and mediastinal lymphadenopathy
Howell Jolly bodies
Hodgkin
Ewing sarcoma
GpIIb/IIIa

5. What is the treatment for lead poising?
Multiple myeloma
Deficiency in factor VIII
B12 in fatty acid pathways leads to subacute combined degeneration
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

6. Which enzyme converts vit k to activated vit k - and what substance inhibits this enzyme
Epoxide reductase - warfarin inhibits
Folate def
Malaria - Babesia
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

7. What happens in beta thal major?
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Inc vasodiltion - inc perm - inc pain
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Leukemia

8. Crew cut on skull xray due to marrow supression - 8% of AA population - single amino acid replacement in beta chain at position 6 - after a DNA point mutation
EBV
Varying shapes
Hb Barts - gamma4 in defect in all 4 alpha genes
Sickle cell

9. What are the extrinsic hemolytic normocytic anemias?
...
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Lymphoma
Hairy cell leukemia

10. What the alpha granules contain in platelets?
Hodgkin
G6PD
VWF and fibrinogen
G6PD

11. What do you see in vit K def
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Beta chain underproduced - asymptomatic - heterozygote
Eleveated PT - PTT
Both alpha and beta thal

12. What is the clinical picture of hemophilia A or B
ADP and Ca
Cromolyn sodium
Macrohemorrhage - hemarthroses - easy bruising
Rhogam - Rh antigen immunoglobulin

13. What is contained within the azurophilic granules of PMNs
Glucose and heme - which inhibit ALA synthase
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Africa = Jaw lesion - US = pelvis or abdomen
Lead - and EtOH

14. What do plasma cells do
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Produce antibody - lots of RER and golgi
Paraprotein spike - monoclonal protein
Megakaryocytes

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

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16. What do auer rods stain with
B12 def
Multiple myeloma
Malaria - Babesia
Peroxidase

17. What is a blast crisis
Hydroxyurea - bone marrow transplant
CML to AML or All
Exposed collagen upon endothelial damage
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

18. What is the result of thrombocytopenia or platelet dysfunction?
Petechiae
Low in CML
Helps platelts adhere to endothelium
Acts to stabilize platelet plug

19. What is the pathogenesis of aplastic anemia with kidney disease
Inc HbF and dec HbS
Intravascular
Schistocytes and inc LDH
Decrease EPO

20. What does CRAB stand for in multiple myeloma
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
CML to AML or All
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Lysosomes

21. What is the difference of presentation of Burkitt in Africa vs the United States
Africa = Jaw lesion - US = pelvis or abdomen
Splicing sites and promotor sequences
IFN gama
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

22. Which substrates begin the heme synthesis pathway
Glycine and succinyl - coa
Porphobilinogen deaminase aka uroporphyrinogen I synthase
CLL (SLL without the peripheral lymphocytosis
Degrades fibrin mesh and converts C3 to C3a

23. What is the pattern of involvement and spread for nonHod lympho
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Schistocytes - helmet cells
Decrease EPO
Lysine for glutamate at position 6

24. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Tissue
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Sideroblastic anemia
Langerhans cell histiocytosis

25. How do platelet disorders present?
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
CML
Plummer - vinson syndrome
Blasts > 5%

26. CD5+ - poor prognosis - t(11;14)
Mantle cell - older males
Inc vasodiltion - inc perm - inc pain
Blod - tissue - MACS
Epoxide reductase - warfarin inhibits

27. hemolytic in a newborn - dec ATP and rigid RBCs
B cells
No lytic bone lesions in WM
Helps platelts adhere to endothelium
Pyruvate kinase def - extravascular

28. What does bradykinin do?
Glycine and succinyl - coa
Factor V resistant to activated protein C's inhibition
Inc vasodiltion - inc perm - inc pain
GpIIb/IIIa

29. Serum iron - transferrin - ferritin lab values for pregs - OCP use
HIV or immunosupression
Antigen - antibody
Epoxide reductase - warfarin inhibits
Nl - up - nl

30. Plasma cell neoplasm
Multiple myeloma
Imatinib - anti bcr abl
120 days
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

31. What is makes a leukemia acute
Leukemia
Blasts > 5%
Ewing sarcoma
Multiple myeloma

32. Who are the professional APCs?
Dendritic cells?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

33. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Hereditary spherocytosis - autoimmune hemolysis
Induces differentiation of myeloblasts
20 to 40

34. What state is commonly associated with nonHod lymphoma
Nl PT - elevated PTT - intrinsic pathway defect
Orotic aciduria
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
HIV or immunosupression

35. Basophilica stippling
Protoporphyrin (blood)
Aspirin
Eleveated PT - PTT
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning

36. Where do you see hypersegmented polys?
Degrades fibrin mesh and converts C3 to C3a
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
B12/folate def

37. What is a metabolic disorder tht can cause macrocytic anemia?
Orotic aciduria
Hb Barts - gamma4 in defect in all 4 alpha genes
Complications of sickle cell anemia
Varying sizes

38. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Polycythemia vera
Lysosomes
Abciximab
All

39. 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
Inc UCB
ADP and Ca
Beta 4

40. What is the most common hodgkin lymphoma
B6 therapy (pyrodixine)
Yes - via MHC II
Plummer - vinson syndrome
Nodular sclerosing

41. Why does B12 def cause neuro sx?
B12 in fatty acid pathways leads to subacute combined degeneration
Follicular lymphoma - indolent course
B6 therapy (pyrodixine)
TRAP (tartrate resistant acid phosphatase

42. What is the treatment for acute intermittent porphyria
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
20 to 40
Glucose and heme - which inhibit ALA synthase

43. What are the pro aggregation factors?
Inc UCB
Beta 4
Acts to stabilize platelet plug
TXA2 - dec blood flow - inc platelet aggregation

44. How are the nucleus and the cytoplasm characterized for lymphocytes
Round densly staining nucleus with a small amount of pale cytoplasm
T(12;21)
VWD
Blasts > 5%

45. What virus can cause an aplastic crisis in pts with HS?
B cells
Paroxysmal nocturnal hemoglobinuria
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Parvovirus

46. What is the effect of ACE on bradykinin
Degrades fibrin mesh and converts C3 to C3a
Inactivates it
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
PGI2 - NO inc blood flow - dec platelet aggregation

47. Upregulated growth of leukocytes in bone marro
20 to 40
Leukemia
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Lymphoma

48. poikilocytosis
Inc UCB
Thrombotic thrombocytopenic purpura
Her next Rh+ fetus
Varying shapes

49. What are the age ranges for the various leukemias
Degrades fibrin mesh and converts C3 to C3a
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
AB - no antibodies
IFN gama

50. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Lacunar in nodular sclerosing variant
Mycosis fundgoides/Sezary syndrome
Protoporphyrin
Folate def