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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. inc WBC count with left shift - inc leukocyte alkaline phosphatase - often due to infection
Rhogam - Rh antigen immunoglobulin
Leukemoid reaction
B12/folate def
Direct coombs - agglutinate if RBCs are coated with Ig

2. What does decreasing heme do to ALA synthase activity?
Inc
Hydroxyurea - bone marrow transplant
Produce antibody - lots of RER and golgi
Eleveated PT - PTT

3. What do you see a starry sky appearance in Burkitt
Thrombotic thrombocytopenic purpura
DIC
Sheets of lymphocytes interspersed with macs
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

4. What reveresible things can a sideroblastic anemia
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Lead - and EtOH
Nodular sclerosing
No platelet clumping

5. What do the iron studies show in sideroblastic anemia
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Fe def - thal - pb poison - sideroblastic anemia
Inc serum iron - normal TIBC - inc ferratin
40 to 50

6. Deficiency in GpIIb/IIIa

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7. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Macrohemorrhage - hemarthroses - easy bruising
Nl - up - nl
Blod - tissue - MACS
Follicular lymphoma - indolent course

8. What is the tx for vWD
Complications of sickle cell anemia
Uroporphyrinogen decarboxylase
HbSS
DDAVP (desmopressin) which releases stored vWF stored in endothelium

9. hypersegmented PMNs - glossitis - dec B12 - inc homocysteine - inc methymalonic acid
Fc
B12 def
DIC
GpIIb/IIIa

10. Bone pain plus anemia
IgG - Warm is GREAT
Multiple myeloma
Schistocytes and inc LDH
Bernard soulier

11. Mutation in 3' untranslated region associated with venous clots
Prothrombin gene mutation
VWF and fibrinogen
Dec plasma volume
G6PD

12. What is the purpose of the fibrin mesh
Pos
Acts to stabilize platelet plug
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Extravascular

13. What does the large SA:volume ratio in RBCs help facilitate?
Leukemoid reaction
Down - down - up
Tissue
Easy gas exchange

14. inc HbF on electrophoresis
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
TPA - cleavage of fibrin mesh
Both alpha and beta thal
Sideroblastic anemia

15. S-100 and CD1a with birbeck granules
M3 AML (acute promyelocytic leukemia)
Langerhans cell histiocytosis
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Produce antibody - lots of RER and golgi

16. What are some causes of cold agglutinin anemia
Glucose and heme - which inhibit ALA synthase
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Dendritic cells?
Produce antibody - lots of RER and golgi

17. What does ADP do?
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
Leukemia
Helps platelts adhere to endothelium
Ankryin - band 4.1 or spectrin

18. What is the enzyme defect in Porphyria cutanea tarda
Uroporphyrinogen decarboxylase
Ticlopidine/clopidogrel
Fibrinogen
Kids= exposure to lead paint - adults = battery - ammunition factory

19. Tdt+ - CAllA+ - kids in the marrow - adolescent males with mediastinal mass
B12 def
All
Collagen - BM - activated platelets
Hydroxyurea - bone marrow transplant

20. What finding you do you see in patients after splenectomy
Factor V resistant to activated protein C's inhibition
Glycine and succinyl - coa
Howell Jolly bodies
AML

21. poikilocytosis
Varying shapes
Low O2 in papilla; can also get microhematuria from medullary infarcts
Contiguous
Direct coombs - agglutinate if RBCs are coated with Ig

22. What is the tx for aplastic anemia
HbSS
EBV
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Tissue

23. How does vWD cause elevated PTT?
Sheets of lymphocytes interspersed with macs
VWF carries/protects factor VIII
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Varying shapes

24. What is the treatment to prevent a woman from forming anti Rh antibody?
Rhogam - Rh antigen immunoglobulin
Up - down - up
Helps platelts adhere to endothelium
Extravascular hemolysis

25. Where to T cells arise and mature - and migrate to...
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Protein S - cleaves and invactivates Va and VIIIa
Adult T cell lymphoma - presents with cutaneous lesions
Bone marrow - thymus - blood (80% of circulating lymphos are T)

26. What substance is Fe added to to yield heme
CML
Protoporphyrin
B cells
Inc HbA2 on electrophoresis

27. t(11;14)
Mantle cell lymphoma
Tissue
Birbeck granules
Hereditary spherocytosis

28. X linked - dec glutathione inc RBC susceptibility to oxidative stress
Protoporphyrin (blood)
G6PD
Histamine - heparin - and eosinohil chemotactic factors
CLL (SLL without the peripheral lymphocytosis

29. hypocellular bone marrown tih fatty infiltration
Prothrombin gene mutation
Aplastic anemia - pancytopenia
Factor V resistant to activated protein C's inhibition
Glanzmann's throbmasthenia

30. What role does antithrombin play?
Incactivates II - VII - IX - X - XI - XII
90% anearobically from glucose to lactate - 10% from HMP shunt
IFN gama
Adult T cell lymphoma - presents with cutaneous lesions

31. deficiency in ADAMTS13 leading to dec degradation of vWF
Nodular sclerosing
Thrombotic thrombocytopenic purpura
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
B6

32. What is the coombs test results in AIHA?
Extrinsic - I - II - V - VII and X
Histaminase and arylsulfatase
Pos
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

33. What is the most common hodgkin lymphoma
Nodular sclerosing
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
40 to 50
Yes - via MHC II

34. What does plasmin do?
Degrades fibrin mesh and converts C3 to C3a
Produce antibody - lots of RER and golgi
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
CLL (SLL without the peripheral lymphocytosis

35. What is the main source of energy in RBCs
90% anearobically from glucose to lactate - 10% from HMP shunt
Sickle cell
Mycosis fundgoides/Sezary syndrome
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

36. What substance prevents mast cells degranulation?
Dec
Porphobilinogen - delta ALA - uroporphyrin
Cromolyn sodium
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII

37. What is the life spance of a platelet?
8-10 days
Ankryin - band 4.1 or spectrin
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Intravascular hemolysis

38. Is HS extravascular or intravascular?
Extravascular
GpIIb/IIIa
Von Willebrand's disease
Lysosomes

39. Back pain - hemoglobinuria
Petechiae
Fc
Orotic aciduria
Common a few days after oxidative stress in a pt with G6PD

40. dec ability to activate factors V and VIII - inc risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
Intravascular hemolysis
Von Willebrand's disease
CD15 and CD30 pos
Protein C or S deficiency

41. What is the characteristic histologic finding in Hodgkin Lymphoma
ATIII def
Easy gas exchange
VWF and fibrinogen
Reed - sternberg cells

42. What CD molecules are on RS cells
Blod - tissue - MACS
Leukemoid reaction
CD15 and CD30 pos
Lysosomes

43. What does NAACP stand for - in regards to the causes of eosinophiia?
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
MAHA
Pyruvate kinase def - extravascular
All trans retinoic acid

44. What is the treatment for acute intermittent porphyria
Glucose and heme - which inhibit ALA synthase
TXA2 - dec blood flow - inc platelet aggregation
Paraprotein spike - monoclonal protein
Fc

45. t(8;14) c - myc gene
Helps platelts adhere to endothelium
Liver disease - abetalipoproteinemia - acntho = spiny
Burkitt lymphoma
Beta chain underproduced - asymptomatic - heterozygote

46. What are the pro aggregation factors?
Varying shapes
Inc
TXA2 - dec blood flow - inc platelet aggregation
Extravascular

47. What are the etiologies of folate def
Howell - Jolly bodies - hypo/asplenia
M3 AML (acute promyelocytic leukemia)
Decrease EPO
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy

48. Which immunoglobulin is involved in warm agglutination?
Bimodal - men>women - except for nodular sclerosing type
Inc RBC - dec O2 sat - inc EPO
IgG - Warm is GREAT
DIC

49. What chromosomal translocation is associated with a better prognosis in All
B6 therapy (pyrodixine)
Hydroxyurea - bone marrow transplant
T(12;21)
Bernard soulier

50. What is the therapy for the M3 variant?
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
All trans retinoic acid
Glucose and heme - which inhibit ALA synthase
Schistocytes and inc LDH