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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 does antithrombin do and What activates it?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Bimodal - men>women - except for nodular sclerosing type

2. What is the mutation in HbS
8-10 days
TXA2 - dec blood flow - inc platelet aggregation
Valine for glutamate
Off center nuclues - clock face chromatin

3. What is the Ddx for nonhemolytic normocytic anemia
B6 therapy (pyrodixine)
Round densly staining nucleus with a small amount of pale cytoplasm
Indirect coombs - agglutinate if serum anti RBC surface Ig
Anemia of chronic disease - Aplastic anemia - kidney disease

4. Which maternal antibodies cross the placenta - anti A/B or anti Rh
Anti Rh
Up - down - nl
Kids= exposure to lead paint - adults = battery - ammunition factory
DIC - TTP/HUS - traumatic hemolysis

5. What is the defect in beta thal?
Fibrinogen
Splicing sites and promotor sequences
Mycosis fundgoides/Sezary syndrome
SLE - CLL - alpha methyldopa

6. normal RBCs added to patient's serum
Mycosis fundgoides/Sezary syndrome
Lung disease - congenital heart diseaes - and high altitude
Howell Jolly bodies
Indirect coombs - agglutinate if serum anti RBC surface Ig

7. In hemophilia A or B What do you see in the coag tests
TXA2 - dec blood flow - inc platelet aggregation
GpIIb/IIIa
Ticlopidine/clopidogrel
Nl PT - elevated PTT - intrinsic pathway defect

8. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Hereditary spherocytosis
Folate def
CML to AML or All
Sideroblastic anemia

9. What does plasmin do?
Degrades fibrin mesh and converts C3 to C3a
Exposed collagen upon endothelial damage
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF

10. What symptoms are associated with Hodgkin lymphoma
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
DIC
Dec synthesis of factors 1972 - protein C/S
B symptoms - fever night sweats - weight loss

11. What does increasing heme do to ALA synthase activity
Dec
TPA - cleavage of fibrin mesh
Bimodal - men>women - except for nodular sclerosing type
Nodular sclerosing

12. Which pathway and factors are tested in the PTT test
Deficiency in factor VIII
Intrinsic - all factors except - VII - XIII
TXA2 - dec blood flow - inc platelet aggregation
Blood

13. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Ringed sideronblasts with iron laden mitochondria
VWD
A antigen and B antibodies
Africa = Jaw lesion - US = pelvis or abdomen

14. What are the pro aggregation factors?
Splicing sites and promotor sequences
ADP and Ca
Both alpha and beta thal
TXA2 - dec blood flow - inc platelet aggregation

15. Blood type B
20 to 40
B antigena and A antibodes
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Folate def

16. Marrow expansion - 'crew cut' on skull x ray - skeletal deformaties - and chipmunk faces
MGUS - monoclonal gammopathy of undetermined significance
PGI2 - NO inc blood flow - dec platelet aggregation
Cromolyn sodium
Beta thal

17. What causes hydrops fetalis
Macrohemorrhage - hemarthroses - easy bruising
Pos
Hb Barts - gamma4 in defect in all 4 alpha genes
Paraprotein spike - monoclonal protein

18. Can B cells function as APCs?
Inc HbF and dec HbS
Beta 4
Nl PT - elevated PTT - intrinsic pathway defect
Yes - via MHC II

19. Serum iron - transferrin - ferritin lab values for pregs - OCP use
PGI2 - NO inc blood flow - dec platelet aggregation
Nl - up - nl
...
Eleveated PT - PTT

20. What is the life spance of a platelet?
Inc
8-10 days
Inactivates it
Common a few days after oxidative stress in a pt with G6PD

21. What is the life span of a normal RBC
Ringed sideronblasts with iron laden mitochondria
120 days
Bone marrow infiltration - myelofibrosis
Inc UCB

22. Schisotcyte - helmet cell
G6PD
DIC - TTP/HUS - traumatic hemolysis
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Reed - sternberg cells

23. What is the age breakdown for hodgkins
Bimodal - men>women - except for nodular sclerosing type
Schistocytes and inc LDH
Howell - Jolly bodies - hypo/asplenia
Schistocytes - helmet cells

24. What are some causes of cold agglutinin anemia
HbSS
Orotic aciduria
Reed - sternberg cells
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

25. deficiency in ADAMTS13 leading to dec degradation of vWF
B cells
Positive osmootic fragility test and splenectomy
Thrombotic thrombocytopenic purpura
Bernard soulier

26. What state is commonly associated with nonHod lymphoma
Down - up - down
Intravascular hemolysis
Mantle cell lymphoma
HIV or immunosupression

27. t(11;14)
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Glycine and succinyl - coa
Ferrocheletase and ALA dehydrogenase
Mantle cell lymphoma

28. Is G6PD intravascular or extravascular
Intravascular
Collagen - BM - activated platelets
Low O2 in papilla; can also get microhematuria from medullary infarcts
CD15 and CD30 pos

29. What virus can cause an aplastic crisis in pts with HS?
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
Parvovirus
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
Decrease EPO

30. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Fibrinogen
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Aplastic anemia - pancytopenia
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

31. What are the labs and tx for HS?
Protein C or S deficiency
90% anearobically from glucose to lactate - 10% from HMP shunt
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Positive osmootic fragility test and splenectomy

32. What is the most common hodgkin lymphoma
Nodular sclerosing
GpIIb/IIIa
AB - no antibodies
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

33. Basophilica stippling
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Extravascular
Common a few days after oxidative stress in a pt with G6PD
Liver disease - abetalipoproteinemia - acntho = spiny

34. When is the peak incidence for nonHod lymphoma
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Lacunar in nodular sclerosing variant
20 to 40

35. inhibition of ferrochelatase and ALA dehydrogenase leading to dec heme synthesis - also inhibition of rRNA degradation
Nodular sclerosing
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Nl PT - elevated PTT - intrinsic pathway defect
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

36. tennis rackets on EM
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
MHC II and Fc receptors
Birbeck granules

37. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Alpha thal - asian and african american
Uroporphyrin (tea colored urine)
Fc
Schistocytes and inc LDH

38. What causes the jaundice in extravascular hemolysis
Von Willebrand's disease
Off center nuclues - clock face chromatin
Inc UCB
Dec synthesis of factors 1972 - protein C/S

39. How are plasma cells characterized?
Fc
Off center nuclues - clock face chromatin
Def in factor IX
Dec plasma volume

40. drug that inhibits ADP induced expression of GpIIb/IIIa
Blasts > 5%
Ticlopidine/clopidogrel
Langerhans cell histiocytosis
Both alpha and beta thal

41. Ringed sideroblasts
Sideroblastic anemia
Common a few days after oxidative stress in a pt with G6PD
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Varying shapes

42. Does CML have a JAK2 mut
Von Willebrand's disease
No
120 days
Inc RBC - dec O2 sat - inc EPO

43. Who has more severe disease - HbSS or HbSC
Extravascular
No platelet clumping
HbSS
Contiguous

44. Basophilic nuclear remnants fonud in RBCs
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Inc lymphoctes - and less RS cells
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Howell - Jolly bodies - hypo/asplenia

45. What activates protein C and What does activated protein C do?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
TPA - cleavage of fibrin mesh
Protein S - cleaves and invactivates Va and VIIIa
Fibrinogen

46. What is the philadelphia chromosome
DIC
Peroxidase
T(9;22) bcr abl
5- FU - AZT - hydroxyurea

47. Which infections can cause MAHA?
Polycythemia vera
Birbeck granules
Malaria - Babesia
HbSS

48. What is the treatment for sideroblastic anemia
T(12;21)
B6 therapy (pyrodixine)
Myelofibrosis (marow is crying being its fibrosed'
Extravascular hemolysis

49. What is the most common nonhodgkin lymphoma
Diffuse Large b cell lymphoma
Aplastic anemia - pancytopenia
Incactivates II - VII - IX - X - XI - XII
Extravascular

50. What is the characteristic histologic finding in Hodgkin Lymphoma
Reed - sternberg cells
Plasma cell
Round densly staining nucleus with a small amount of pale cytoplasm
Indirect coombs - agglutinate if serum anti RBC surface Ig