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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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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. What is the age breakdown for hodgkins
Langerhans cell histiocytosis
Bimodal - men>women - except for nodular sclerosing type
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Common a few days after oxidative stress in a pt with G6PD

2. Who are the professional APCs?
Pyruvate kinase def - extravascular
Inc serum iron - normal TIBC - inc ferratin
Dendritic cells?
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

3. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
No lytic bone lesions in WM
Reed - sternberg cells
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

4. inc LDH - jaundice
Blistering cutaneous photosens - most common porphyria
Kids= exposure to lead paint - adults = battery - ammunition factory
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Extravascular hemolysis

5. What CD molecules are on RS cells
CD15 and CD30 pos
Protein C or S deficiency
Factor V resistant to activated protein C's inhibition
Nl PT - elevated PTT - intrinsic pathway defect

6. Who has more severe disease - HbSS or HbSC
Fibrinogen bind GpIIb/IIIa and links platelts
HbSS
Signs and sx of aplastic anemia
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

7. What is the mutation in HbC
Lysosomes
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
EBV
Lysine for glutamate at position 6

8. What is the treatment for lead poising?
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
B12 def
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

9. Which pathway and factorrs are tested by the PT coag test
Mantle cell - older males
Extrinsic - I - II - V - VII and X
Langerhans cells
Tissue

10. What is the mutation in HbS
Schistocytes - helmet cells
Ewing sarcoma
Fibrinogen bind GpIIb/IIIa and links platelts
Valine for glutamate

11. Basophilic nuclear remnants fonud in RBCs
5- FU - AZT - hydroxyurea
Howell - Jolly bodies - hypo/asplenia
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Anti Rh

12. What substance accumulates in porphyria cutanea
Uroporphyrin (tea colored urine)
Down - up - down
Hodgkin
90% anearobically from glucose to lactate - 10% from HMP shunt

13. How do platelet disorders present?
Varying sizes
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Von Willebrand's disease
Inc lymphoctes - and less RS cells

14. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Low O2 in papilla; can also get microhematuria from medullary infarcts
Paroxysmal nocturnal hemoglobinuria
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Bleeding - malnutrition/absorption - inc demand pregs

15. What are dendritic cells called in the skin?
Langerhans cells
M3 AML (acute promyelocytic leukemia)
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
EBV

16. What begins the extrinsic pathway?
Lung disease - congenital heart diseaes - and high altitude
No lytic bone lesions in WM
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
Tissue factor converst the VII and VIIa

17. What are the likely exposures of kids and adults for lead poisoning
Burkitt lymphoma
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Kids= exposure to lead paint - adults = battery - ammunition factory
SLE - CLL - alpha methyldopa

18. drug that inhibits ADP induced expression of GpIIb/IIIa
Ticlopidine/clopidogrel
T(9;22) bcr abl
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Factor V resistant to activated protein C's inhibition

19. Which factor does vWF carry/protect
VIII
Bernard soulier
Easy gas exchange
Langerhans cells

20. What is the tx for sickle cell
Down - down - up
Cromolyn sodium
Hydroxyurea - bone marrow transplant
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

21. Acanthocyte (spur cell)
Aspirin
Liver disease - abetalipoproteinemia - acntho = spiny
Mycosis fundgoides/Sezary syndrome
MHC II and Fc receptors

22. What does CRAB stand for in multiple myeloma
EBV
TPA - cleavage of fibrin mesh
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Helps platelts adhere to endothelium

23. What percentage of WBCs are eosinophils?
1 to 6
Mantle cell - older males
Alpha thal - asian and african american
Lung disease - congenital heart diseaes - and high altitude

24. anti - GpIIb/IIIa antibodies leading to peripheral platelet destruction
Hereditary spherocytosis
Idiopathic thrombocytopenia (ITP)
Protoporphyrin
Nl - up - nl

25. What does the blood smear show in glanzmann's?
Abciximab
Induces differentiation of myeloblasts
HbSS
No platelet clumping

26. Where are mast cells found
Alpha thal - asian and african american
Tissue
Malaria - Babesia
ADP and Ca

27. Deficiency in GpIIb/IIIa

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28. universal recipient
AB - no antibodies
Myelofibrosis (marow is crying being its fibrosed'
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Peroxidase

29. What activates protein C and What does activated protein C do?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Mantle cell - older males
B6
Protein S - cleaves and invactivates Va and VIIIa

30. Where does All spread
Inactivates it
IFN gama
Down - down - up
CNS and testis

31. Elliptocyte
B symptoms - fever night sweats - weight loss
Epoxide reductase - warfarin inhibits
Her next Rh+ fetus
Hereditary elloptocytosis

32. Eosinophils are highly phagocytic For what kind of complex?
IgG - Warm is GREAT
EBV
MAHA
Antigen - antibody

33. Blood type B
Leukemoid reaction
Tissue factor converst the VII and VIIa
90% anearobically from glucose to lactate - 10% from HMP shunt
B antigena and A antibodes

34. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Von Willebrand's disease
VIII
Sideroblastic anemia
Hereditary spherocytosis - autoimmune hemolysis

35. What is HbH
Beta 4
Epoxide reductase - warfarin inhibits
GpIIb/IIIa
Idiopathic thrombocytopenia (ITP)

36. Target cell

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37. What state is commonly associated with nonHod lymphoma
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Blod - tissue - MACS
HIV or immunosupression
MAHA

38. universal donor
No antigen - both antibodies
Folate def
Contiguous
Mantle cell - older males

39. What the alpha granules contain in platelets?
VWF and fibrinogen
Sickle cell anemia
Intravascular hemolysis
Pos

40. What do the dense granules contain in platelets
Blistering cutaneous photosens - most common porphyria
Paraprotein spike - monoclonal protein
Dec plasma volume
ADP and Ca

41. What are the extrinsic hemolytic normocytic anemias?
...
Her next Rh+ fetus
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
VWD

42. What do labs show in ITP?
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Varying sizes
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

43. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Blasts > 5%
Antigen - antibody
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

44. Why can newborns with sickle cell be asymptomatic
Dendritic cells?
Down - down - up
Inc large vWF multimers - inc platelet aggregation and thrombosis
Inc HbF and dec HbS

45. What is the clinical picture of hemophilia A or B
Lymphoma
Macrohemorrhage - hemarthroses - easy bruising
B12 in fatty acid pathways leads to subacute combined degeneration
No antigen - both antibodies

46. What is the characteristic histologic finding in Hodgkin Lymphoma
Fibrinogen bind GpIIb/IIIa and links platelts
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Reed - sternberg cells
Collagen - BM - activated platelets

47. What is the affected enzyme in acute intermittment porphyria
Hairy cell leukemia
Easy gas exchange
Protein C or S deficiency
Porphobilinogen - delta ALA - uroporphyrin

48. What is relative polycythemia
Dec plasma volume
Lysine for glutamate at position 6
Inc vasodiltion - inc perm - inc pain
A antigen and B antibodies

49. Blood type A
A antigen and B antibodies
Protein S - cleaves and invactivates Va and VIIIa
Howell Jolly bodies
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

50. discrete tumor masses arising from lymph nodes
Up - down - nl
B antigena and A antibodes
Lymphoma
Direct coombs - agglutinate if RBCs are coated with Ig