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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. hypocellular bone marrown tih fatty infiltration
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 -
Antigen - antibody
Inc lymphoctes - and less RS cells
Aplastic anemia - pancytopenia

2. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Lysosomes
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
T(12;21)
Alpha thal - asian and african american

3. What does antithrombin do and What activates it?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
T(9;22) bcr abl
Fibrinogen bind GpIIb/IIIa and links platelts
Mycosis fundgoides/Sezary syndrome

4. Who are the professional APCs?
Birbeck granules
Dec synthesis of factors 1972 - protein C/S
Dendritic cells?
EBV

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

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6. What is the affected enzyme in acute intermittment porphyria
DDAVP (desmopressin) which releases stored vWF stored in endothelium
Yes - via MHC II
TRAP (tartrate resistant acid phosphatase
Porphobilinogen - delta ALA - uroporphyrin

7. Which pathway and factors are tested in the PTT test
Aplastic anemia - pancytopenia
Contiguous
Low O2 in papilla; can also get microhematuria from medullary infarcts
Intrinsic - all factors except - VII - XIII

8. What causes the physiologic chloride shift and What does the chloride shift do?
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Ankryin - band 4.1 or spectrin
Inc HbA2 on electrophoresis
Nl PT - elevated PTT - intrinsic pathway defect

9. anti - Ig antibody added to patients RBCs;
Direct coombs - agglutinate if RBCs are coated with Ig
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Beta thal

10. S-100 and CD1a with birbeck granules
Porphobilinogen - delta ALA - uroporphyrin
Bleeding - malnutrition/absorption - inc demand pregs
Protoporphyrin (blood)
Langerhans cell histiocytosis

11. What happens in beta thal major?
Contiguous
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
5- FU - AZT - hydroxyurea
Ferrocheletase and ALA dehydrogenase

12. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
Extravascular hemolysis
Complications of sickle cell anemia
Beta thal
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'

13. What are the anti aggregation factors?
Lead - and EtOH
PGI2 - NO inc blood flow - dec platelet aggregation
SLE - CLL - alpha methyldopa
Liver disease - abetalipoproteinemia - acntho = spiny

14. Where do B cells arise from - mature - and migrate to...
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Valine for glutamate
Inc HbF and dec HbS
Lymphoma

15. What vitamin is a cofactor for the first step of heme synthesis
Epoxide reductase - warfarin inhibits
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
B6
Lymphoma

16. What is appropriate absolyte polycythemia associated with
Fc
Macrohemorrhage - hemarthroses - easy bruising
Ferrocheletase and ALA dehydrogenase
Lung disease - congenital heart diseaes - and high altitude

17. How are plasma cells characterized?
Off center nuclues - clock face chromatin
CML to AML or All
Bite cells and Heinz bodies
Plasma cell

18. Spherocyte
Hereditary spherocytosis - autoimmune hemolysis
Off center nuclues - clock face chromatin
Abciximab
Protein C or S deficiency

19. What is the Ddx for a normocytic - normochromic anemia?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Ringed sideronblasts with iron laden mitochondria
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

20. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Dendritic cells?
Fibrinogen
Low in CML

21. What is the treatment for lead poising?
Prothrombin gene mutation
Nodular sclerosing
Aplastic anemia - pancytopenia
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

22. CD5+ - poor prognosis - t(11;14)
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Bone marrow infiltration - myelofibrosis
Degrades fibrin mesh and converts C3 to C3a
Mantle cell - older males

23. what proteins can be defective in HS?
Ankryin - band 4.1 or spectrin
DIC
Inc HbA2 on electrophoresis
5- FU - AZT - hydroxyurea

24. Elderly - mature b cell tumor with filamentous - hairlike projections
Hairy cell leukemia
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Langerhans cells
Sickle cell

25. What state is commonly associated with nonHod lymphoma
Intravascular
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
HIV or immunosupression
ADP and Ca

26. Which maternal antibodies cross the placenta - anti A/B or anti Rh
MAHA
Round densly staining nucleus with a small amount of pale cytoplasm
Anti Rh
Uroporphyrin (tea colored urine)

27. What chromosomal translocation is associated with a better prognosis in All
T(12;21)
Porphyrias
TPA - cleavage of fibrin mesh
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

28. What is the pathogenesis of ACD?
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
Polycythemia vera
Down - up - down
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin

29. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Her next Rh+ fetus
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Porphobilinogen - delta ALA - uroporphyrin
Cromolyn sodium

30. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
VWD
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
They bind vWF via GpIb
90% anearobically from glucose to lactate - 10% from HMP shunt

31. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Inc
Beta chain underproduced - asymptomatic - heterozygote
Inc vasodiltion - inc perm - inc pain
Protein C or S deficiency

32. What is the characteristic histologic finding in Hodgkin Lymphoma
Bone marrow infiltration - myelofibrosis
MGUS - monoclonal gammopathy of undetermined significance
Reed - sternberg cells
Schistocytes and inc LDH

33. What is the purpose of the fibrin mesh
Peroxidase
All trans retinoic acid
Inc RBC - dec O2 sat - inc EPO
Acts to stabilize platelet plug

34. Ddx for microcytic anemia
Decrease EPO
Intravascular hemolysis
Fe def - thal - pb poison - sideroblastic anemia
Birbeck granules

35. What substance prevents mast cells degranulation?
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Varying shapes
Cromolyn sodium
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread

36. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
AML
Pyruvate kinase def - extravascular
Sideroblastic anemia
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

37. What does the blood smear show in glanzmann's?
Round densly staining nucleus with a small amount of pale cytoplasm
No platelet clumping
DIC
GpIIb/IIIa

38. abnormal clone of hematopoetic stem cells are increasingly sensitive to growth factors - inc RBC - JAK2 mut
Helps platelts adhere to endothelium
Protoporphyrin (blood)
Polycythemia vera
Gp1b

39. t(11;14)
Antigen - antibody
Petechiae
Mantle cell lymphoma
Intravascular

40. What activates the fibrinolytic pathway?
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
AB - no antibodies
Glucose and heme - which inhibit ALA synthase

41. What is the mutation in HbS
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
All trans retinoic acid
Valine for glutamate
Inducers of primary antibody response

42. What percentage if WBCs are polys?
M3 AML (acute promyelocytic leukemia)
Ringed sideronblasts with iron laden mitochondria
Intrinsic - all factors except - VII - XIII
40 to 75

43. Target cell

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44. What causes the jaundice in extravascular hemolysis
Inc lymphoctes - and less RS cells
B antigena and A antibodes
Inc UCB
Kids= exposure to lead paint - adults = battery - ammunition factory

45. What is the general pathology of a macrocytic anemia?
Hodgkin
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
VWF and fibrinogen
Schistocytes and inc LDH

46. How are the nucleus and the cytoplasm characterized for lymphocytes
Ticlopidine/clopidogrel
Round densly staining nucleus with a small amount of pale cytoplasm
Inc serum iron - normal TIBC - inc ferratin
Beta 4

47. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Inc UCB
Nl - up - nl
VWF carries/protects factor VIII
Ferrocheletase and ALA dehydrogenase

48. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
Hereditary spherocytosis
Birbeck granules
Varying shapes
B antigena and A antibodes

49. Which drugs can cause macrocytic anemia?
Uroporphyrin (tea colored urine)
ADP and Ca
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
5- FU - AZT - hydroxyurea

50. Blood type A
Examples of oxidative stress
Alpha thal - asian and african american
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
A antigen and B antibodies