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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. What is the aggregation phase of ppf?
Nl PT - elevated PTT - intrinsic pathway defect
Fibrinogen bind GpIIb/IIIa and links platelts
Positive osmootic fragility test and splenectomy
Polycythemia vera

2. What virus is associated with Burkitt lymphoma
Pyruvate kinase def - extravascular
Her next Rh+ fetus
EBV
AML

3. What are the etiologies of B12 def
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Induces differentiation of myeloblasts
Petechiae

4. Spherocyte
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Schistocytes and inc LDH
Hereditary spherocytosis - autoimmune hemolysis
Contiguous

5. Schisotcyte - helmet cell
Hairy cell leukemia
No platelet clumping
DIC - TTP/HUS - traumatic hemolysis
Porphobilinogen deaminase aka uroporphyrinogen I synthase

6. Bone pain plus anemia
Hereditary spherocytosis - G6PD - sickle cell
No lytic bone lesions in WM
Easy gas exchange
Multiple myeloma

7. Is G6PD intravascular or extravascular
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Intravascular
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Degrades fibrin mesh and converts C3 to C3a

8. What is the affected enzyme in acute intermittment porphyria
Uroporphyrin (tea colored urine)
Uroporphyrinogen decarboxylase
Porphobilinogen - delta ALA - uroporphyrin
Lacunar in nodular sclerosing variant

9. What is the affected enzyme in lead poisoning
They bind vWF via GpIb
Leukemia
Ferrocheletase and ALA dehydrogenase
Inc lymphoctes - and less RS cells

10. What do platelets interact with to form a hemostatic plug
Howell Jolly bodies
Fibrinogen
8-10 days
Dec

11. What percentage of WBCs are eosinophils?
Porphobilinogen - delta ALA - uroporphyrin
Direct coombs - agglutinate if RBCs are coated with Ig
1 to 6
Glanzmann's throbmasthenia

12. What is the result of thrombocytopenia or platelet dysfunction?
Petechiae
Inc
Idiopathic thrombocytopenia (ITP)
Ringed sideronblasts with iron laden mitochondria

13. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Howell - Jolly bodies - hypo/asplenia
Paroxysmal nocturnal hemoglobinuria
20 to 40

14. What is the pattern of involvement and spread for nonHod lympho
Produce antibody - lots of RER and golgi
B12 def
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
No antigen - both antibodies

15. Where does All spread
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
CNS and testis
Inc vasodiltion - inc perm - inc pain
Inc HbF and dec HbS

16. Where do B cells arise from - mature - and migrate to...
Multiple myeloma
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Def in factor IX
VWF and fibrinogen

17. What are the four levels of alpha thal?
Plasma cell
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
CD15 and CD30 pos
Induces differentiation of myeloblasts

18. What causes renal papillary necrosis in sickle cell
Low O2 in papilla; can also get microhematuria from medullary infarcts
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
ADP and Ca
IgG - Warm is GREAT

19. What signal activates MACS
G6PD
Prothrombin gene mutation
B12 in fatty acid pathways leads to subacute combined degeneration
IFN gama

20. philadelphia chromosome - blood looks like marrow
Thrombotic thrombocytopenic purpura
CML
MGUS - monoclonal gammopathy of undetermined significance
Sickle cell anemia

21. What activates the intrinsic pathway?
Indirect coombs - agglutinate if serum anti RBC surface Ig
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 -
Collagen - BM - activated platelets
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

22. What causes hydrops fetalis
Ewing sarcoma
Hb Barts - gamma4 in defect in all 4 alpha genes
Varying sizes
Liver disease - abetalipoproteinemia - acntho = spiny

23. What is the pathogenesis of aplastic anemia with kidney disease
Decrease EPO
Factor V Leiden
Dec synthesis of factors 1972 - protein C/S
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

24. What do the platelets bind? What is the step called
They bind vWF via GpIb
Ankryin - band 4.1 or spectrin
Hydroxyurea - bone marrow transplant
Tissue factor converst the VII and VIIa

25. What substances are released in mast cell degranulation
Hydroxyurea - bone marrow transplant
Adult T cell lymphoma - presents with cutaneous lesions
Histamine - heparin - and eosinohil chemotactic factors
Dec synthesis of factors 1972 - protein C/S

26. what proteins can be defective in HS?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Ankryin - band 4.1 or spectrin
No

27. bcl -2 t(14;18) adults
Follicular lymphoma - indolent course
B12/folate def
Both alpha and beta thal
Examples of oxidative stress

28. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
TPA - cleavage of fibrin mesh
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Folate def
Helminth infections major basic protein

29. What do you see in vit K def
Pos
HbSS
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Eleveated PT - PTT

30. What is the therapy for CML (philly chrom)
Myelofibrosis (marow is crying being its fibrosed'
Multiple myeloma
T(12;21)
Imatinib - anti bcr abl

31. Deficiency in vWF

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32. What the alpha granules contain in platelets?
B12/folate def
GpIIb/IIIa
VWF and fibrinogen
Factor V resistant to activated protein C's inhibition

33. Mutation in 3' untranslated region associated with venous clots
B12/folate def
Idiopathic thrombocytopenia (ITP)
No
Prothrombin gene mutation

34. What are the age ranges for the various leukemias
B12 in fatty acid pathways leads to subacute combined degeneration
Dendritic cells?
Produce antibody - lots of RER and golgi
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60

35. What is the most common hodgkin lymphoma
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Bite cells and Heinz bodies
Multiple myeloma
Nodular sclerosing

36. Macro - ovalocyte
Positive osmootic fragility test and splenectomy
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Heinz bodies - seen in alpha thal and G6PD
Von Willebrand's disease

37. What vitamin is a cofactor for the first step of heme synthesis
B6
Fibrinogen bind GpIIb/IIIa and links platelts
Histaminase and arylsulfatase
Protein C or S deficiency

38. What is the effected enzyme in acute intermittment porphyria?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Megakaryocytes
...
Porphobilinogen deaminase aka uroporphyrinogen I synthase

39. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Low in CML
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Paraprotein spike - monoclonal protein
Idiopathic thrombocytopenia (ITP)

40. t(11;14)
VWF and fibrinogen
90% anearobically from glucose to lactate - 10% from HMP shunt
Mantle cell lymphoma
Protein S - cleaves and invactivates Va and VIIIa

41. anti - Ig antibody added to patients RBCs;
20 to 40
Direct coombs - agglutinate if RBCs are coated with Ig
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Protoporphyrin

42. What is the characteristic lab finding on electrophoresis
Myelofibrosis (marow is crying being its fibrosed'
Extravascular hemolysis
Dec
Paraprotein spike - monoclonal protein

43. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
Parvovirus
1 to 6
Down - up - down
Histaminase and arylsulfatase

44. What is the mutation in HbS
Valine for glutamate
TRAP (tartrate resistant acid phosphatase
Inc UCB
Nodular sclerosing

45. How is beta thal minor dx
Collagen - BM - activated platelets
Inc HbA2 on electrophoresis
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Lacunar in nodular sclerosing variant

46. What is factor V leidin?

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47. What converts plasminogen to plasm and What does plasmin do?
TXA2 - dec blood flow - inc platelet aggregation
Deficiency in factor VIII
VWF carries/protects factor VIII
TPA - cleavage of fibrin mesh

48. What are the presenting symptoms of porphyria cutanea tarda
Signs and sx of aplastic anemia
Blistering cutaneous photosens - most common porphyria
Contiguous
Hairy cell leukemia

49. How are the nucleus and the cytoplasm characterized for lymphocytes
AML
Langerhans cell histiocytosis
Round densly staining nucleus with a small amount of pale cytoplasm
Mycosis fundgoides/Sezary syndrome

50. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Reed - sternberg cells
B12 def
Porphobilinogen deaminase aka uroporphyrinogen I synthase
TPA - cleavage of fibrin mesh