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Hemeonc

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Answer 50 questions in 15 minutes.
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1. What is the pattern of involvement and spread for nonHod lympho
Gp1b
Glanzmann's throbmasthenia
Easy gas exchange
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

2. t(11:22)
Ewing sarcoma
Multiple myeloma
B12/folate def
Inc vasodiltion - inc perm - inc pain

3. What percentage of WBCs are basophils - and What is found in their basophilic granules?
B6 therapy (pyrodixine)
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Intravascular
< 1% - heparin - histamine - LTD-4 - other vasoactive amines

4. Ringed sideroblasts
Sideroblastic anemia
Varying sizes
IgG - Warm is GREAT
Hairy cell leukemia

5. What is factor V leidin?

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6. What are the four levels of alpha thal?
Histaminase and arylsulfatase
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Essential thrombocytosis
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

7. Is G6PD intravascular or extravascular
Intravascular
Varying shapes
Inc lymphoctes - and less RS cells
Inc HbF and dec HbS

8. What indicated a good prognosis in Hodgkin lymphoma?
No antigen - both antibodies
Varying sizes
Inc lymphoctes - and less RS cells
Acts to stabilize platelet plug

9. What is the general pathology of a macrocytic anemia?
AB - no antibodies
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
TPA - cleavage of fibrin mesh
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

10. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Intrinsic - all factors except - VII - XIII
CLL (SLL without the peripheral lymphocytosis
G6PD
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion

11. Where do you see hypersegmented polys?
B12/folate def
Megakaryocytes
A antigen and B antibodies
Dec synthesis of factors 1972 - protein C/S

12. What is the pathogenesis of TTP?
Tissue factor converst the VII and VIIa
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Inc large vWF multimers - inc platelet aggregation and thrombosis

13. inc LDH - jaundice
Antigen - antibody
VWF and fibrinogen
Nl PT - elevated PTT - intrinsic pathway defect
Extravascular hemolysis

14. What is the receptor for fibrinogen?
GpIIb/IIIa
Fe def - thal - pb poison - sideroblastic anemia
5- FU - AZT - hydroxyurea
Lysosomes

15. hemolytic in a newborn - dec ATP and rigid RBCs
Follicular lymphoma - indolent course
Imatinib - anti bcr abl
Pyruvate kinase def - extravascular
B6 therapy (pyrodixine)

16. Basophilica stippling
Imatinib - anti bcr abl
Bimodal - men>women - except for nodular sclerosing type
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
DIC - TTP/HUS - traumatic hemolysis

17. What are the pro aggregation factors?
Glycine and succinyl - coa
B12 def
TXA2 - dec blood flow - inc platelet aggregation
Howell Jolly bodies

18. When is the peak incidence for nonHod lymphoma
Exposed collagen upon endothelial damage
CML
Complications of sickle cell anemia
20 to 40

19. What is the treatment for acute intermittent porphyria
Bernard soulier
Anti Rh
Glucose and heme - which inhibit ALA synthase
Fibrinogen bind GpIIb/IIIa and links platelts

20. What is the affected enzyme in acute intermittment porphyria
Lysine for glutamate at position 6
Porphobilinogen - delta ALA - uroporphyrin
Plasma cell
VIII

21. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
G6PD
No lytic bone lesions in WM
Protein C or S deficiency
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

22. drug that inhibits ADP induced expression of GpIIb/IIIa
Glucose and heme - which inhibit ALA synthase
Ticlopidine/clopidogrel
Histamine - heparin - and eosinohil chemotactic factors
Varying sizes

23. What does the blood smear show in glanzmann's?
IgG - Warm is GREAT
No platelet clumping
They bind vWF via GpIb
Down - up - down

24. philadelphia chromosome - blood looks like marrow
Decrease EPO
B6 therapy (pyrodixine)
CML
Inc

25. What CD molecules are on RS cells
Liver disease - abetalipoproteinemia - acntho = spiny
1 to 6
Collagen - BM - activated platelets
CD15 and CD30 pos

26. tennis rackets on EM
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Birbeck granules
Up - down - nl
20 to 40

27. Where do B cells arise from - mature - and migrate to...
Lymphoma
ATIII def
Hereditary spherocytosis
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

28. From what cells are platelets derived from
Protein S - cleaves and invactivates Va and VIIIa
Megakaryocytes
Fibrinogen
TXA2 - dec blood flow - inc platelet aggregation

29. What are the etiologies of B12 def
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
VWF carries/protects factor VIII
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Intrinsic - all factors except - VII - XIII

30. What is the Ddx for nonhemolytic normocytic anemia
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Hydroxyurea - bone marrow transplant
Anemia of chronic disease - Aplastic anemia - kidney disease
Hereditary spherocytosis

31. What condition can result from treating AML M3 from the release of the Auer rods
DIC
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Both alpha and beta thal
Ticlopidine/clopidogrel

32. What do labs show in ITP?
Polycythemia vera
DIC
CLL (SLL without the peripheral lymphocytosis
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

33. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Sideroblastic anemia
Blood
2 to 10
Kids= exposure to lead paint - adults = battery - ammunition factory

34. Bone pain plus anemia
Glucose and heme - which inhibit ALA synthase
Multiple myeloma
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Down - up - down

35. What is the pathogenesis of sickle cell
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Ankryin - band 4.1 or spectrin
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

36. adults - auer rods - inc circulating myeblasts on peripheral smear
Protein S - cleaves and invactivates Va and VIIIa
Leukemoid reaction
Beta 4
AML

37. What is monoclonal expansion without symptoms associated with multiple myeloma?
Histamine - heparin - and eosinohil chemotactic factors
MGUS - monoclonal gammopathy of undetermined significance
Fe def - thal - pb poison - sideroblastic anemia
Degrades fibrin mesh and converts C3 to C3a

38. Megakaryocytosis
Inc large vWF multimers - inc platelet aggregation and thrombosis
Essential thrombocytosis
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Fibrinogen

39. universal donor
Glucose and heme - which inhibit ALA synthase
No antigen - both antibodies
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Helps platelts adhere to endothelium

40. What is the tx for aplastic anemia
Complications of sickle cell anemia
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Off center nuclues - clock face chromatin
Def in factor IX

41. Drug that inhibits COX and therefore TXA2 synthesis
Schistocytes and inc LDH
TRAP (tartrate resistant acid phosphatase
Aspirin
Yes - via MHC II

42. bcl -2 t(14;18) adults
Porphobilinogen - delta ALA - uroporphyrin
Follicular lymphoma - indolent course
Paraprotein spike - monoclonal protein
Sideroblastic anemia

43. What does NAACP stand for - in regards to the causes of eosinophiia?
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
8-10 days
1 to 6
Aspirin

44. What do platelets interact with to form a hemostatic plug
Blood
Fibrinogen
Sideroblastic anemia
Extravascular

45. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
Protein C or S deficiency
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Leukemia
Inc HbA2 on electrophoresis

46. What are the likely exposures of kids and adults for lead poisoning
Kids= exposure to lead paint - adults = battery - ammunition factory
ATIII def
Helminth infections major basic protein
Blasts > 5%

47. Eosinophils are highly phagocytic For what kind of complex?
Hereditary spherocytosis - autoimmune hemolysis
Round densly staining nucleus with a small amount of pale cytoplasm
Protein C or S deficiency
Antigen - antibody

48. deficiency in ADAMTS13 leading to dec degradation of vWF
Abciximab
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Glanzmann's throbmasthenia
Thrombotic thrombocytopenic purpura

49. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Dec synthesis of factors 1972 - protein C/S
Anemia of chronic disease - Aplastic anemia - kidney disease

50. What does 'Neutrophils Like Making Everything Better' stand for?
Collagen - BM - activated platelets
Indirect coombs - agglutinate if serum anti RBC surface Ig
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Produce antibody - lots of RER and golgi

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