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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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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 purpose of the fibrin mesh
Rhogam - Rh antigen immunoglobulin
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
EBV
Acts to stabilize platelet plug

2. What do plasma cells do
Liver disease - abetalipoproteinemia - acntho = spiny
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Bernard soulier
Produce antibody - lots of RER and golgi

3. What is their role?
Tissue
Porphyrias
Inducers of primary antibody response
20 to 40

4. universal recipient
AB - no antibodies
G6PD
B6 therapy (pyrodixine)
Protoporphyrin

5. What does 'Neutrophils Like Making Everything Better' stand for?
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Lead - and EtOH
Uroporphyrinogen decarboxylase
Valine for glutamate

6. Drug that inhibits COX and therefore TXA2 synthesis
VWF and fibrinogen
Von Willebrand's disease
Aspirin
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm

7. What is makes a leukemia acute
Von Willebrand's disease
Blasts > 5%
8-10 days
Helps platelts adhere to endothelium

8. What is the receptor for fibrinogen?
GpIIb/IIIa
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Intrinsic - all factors except - VII - XIII
All

9. bcl -2 t(14;18) adults
B12 def
Splicing sites and promotor sequences
Follicular lymphoma - indolent course
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

10. What state is commonly associated with nonHod lymphoma
HIV or immunosupression
Hydroxyurea - bone marrow transplant
VIII
Dendritic cells?

11. What are some classic examples of extravascular hemolysis
Kids= exposure to lead paint - adults = battery - ammunition factory
Hereditary spherocytosis - G6PD - sickle cell
Porphyrias
Def in factor IX

12. lymphoid neoplasms with widespread involvement of bone marrow - tumor cells usually foind in peripheral blood
EBV
Leukemia
Prothrombin gene mutation
Blod - tissue - MACS

13. Is G6PD intravascular or extravascular
T(12;21)
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Intravascular
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis

14. Spherocyte
Extravascular hemolysis
Hereditary spherocytosis - autoimmune hemolysis
Pyruvate kinase def - extravascular
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs

15. What is the tx for sickle cell
Blood
Blasts > 5%
Hydroxyurea - bone marrow transplant
B antigena and A antibodes

16. What is the mutation in HbS
Bernard soulier
DIC
Valine for glutamate
VWF and fibrinogen

17. What activates the fibrinolytic pathway?
Varying shapes
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

18. X linked - dec glutathione inc RBC susceptibility to oxidative stress
IFN gama
G6PD
Ticlopidine/clopidogrel
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

19. What percentage if WBCs are polys?
Degrades fibrin mesh and converts C3 to C3a
G6PD
5- FU - AZT - hydroxyurea
40 to 75

20. Elliptocyte
Reed - sternberg cells
Hereditary elloptocytosis
DIC
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

21. What do eosinophils defend against and What do they use to do it?
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Helminth infections major basic protein
Birbeck granules
Fc

22. defect in alpha globin gene - dec alpha globin synth - disease and populations?
Alpha thal - asian and african american
Tissue factor converst the VII and VIIa
Valine for glutamate
Thrombotic thrombocytopenic purpura

23. What substance is Fe added to to yield heme
Protoporphyrin
ADP and Ca
B6 therapy (pyrodixine)
Megakaryocytes

24. Deficiency in vWF

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25. Serum iron - transferrin - ferritin lab values for pregs - OCP use
Malaria - Babesia
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Nl - up - nl
Varying sizes

26. What are the variants of the RS cells
Lacunar in nodular sclerosing variant
Protein S - cleaves and invactivates Va and VIIIa
Intravascular
Porphobilinogen - delta ALA - uroporphyrin

27. What portion of IgE can mast cells bind
Uroporphyrin (tea colored urine)
Myelofibrosis (marow is crying being its fibrosed'
Tissue
Fc

28. What is the pathogenesis of sickle cell
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
MHC II and Fc receptors
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Low O2 in papilla; can also get microhematuria from medullary infarcts

29. What does STOP Making New Thrombi stand for
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Hairy cell leukemia
Plummer - vinson syndrome
Schistocytes and inc LDH

30. What are the etiologies of B12 def
Inc
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
VWF and fibrinogen
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium

31. What is the therapy for CML (philly chrom)
Mantle cell - older males
Imatinib - anti bcr abl
Paroxysmal nocturnal hemoglobinuria
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

32. Adults present with cutaneous patches/nodules - indolent CD4+
Mycosis fundgoides/Sezary syndrome
Gp1b
Off center nuclues - clock face chromatin
Round densly staining nucleus with a small amount of pale cytoplasm

33. Where are 1/3 of platelets stored
Blod - tissue - MACS
Lung disease - congenital heart diseaes - and high altitude
Porphobilinogen deaminase aka uroporphyrinogen I synthase
Spleen

34. adults - auer rods - inc circulating myeblasts on peripheral smear
AML
B antigena and A antibodes
Hereditary spherocytosis - G6PD - sickle cell
Leukemoid reaction

35. Plasma cell neoplasm
Bernard soulier
Tissue factor converst the VII and VIIa
Multiple myeloma
Indirect coombs - agglutinate if serum anti RBC surface Ig

36. What is the activation stage of platelet plug formation?
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Porphyrias
Fe def - thal - pb poison - sideroblastic anemia
B symptoms - fever night sweats - weight loss

37. What is the treatment for sideroblastic anemia
Low O2 in papilla; can also get microhematuria from medullary infarcts
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
Inc serum iron - normal TIBC - inc ferratin
B6 therapy (pyrodixine)

38. What converts plasminogen to plasm and What does plasmin do?
Protein C or S deficiency
A antigen and B antibodies
TPA - cleavage of fibrin mesh
Peroxidase

39. What signal activates MACS
Complications of sickle cell anemia
IFN gama
Sheets of lymphocytes interspersed with macs
MGUS - monoclonal gammopathy of undetermined significance

40. Wilm's tumor - RCC - HCC - hydronephrosis
EBV
Ankryin - band 4.1 or spectrin
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO

41. poikilocytosis
Varying shapes
Positive osmootic fragility test and splenectomy
Inactivates it
Leukemia

42. What is the mutation in HbC
Paraprotein spike - monoclonal protein
AB - no antibodies
Lysine for glutamate at position 6
A antigen and B antibodies

43. What is the tx for aplastic anemia
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Thrombotic thrombocytopenic purpura
1 to 6
No lytic bone lesions in WM

44. What is the pathogenesis of ACD?
Complications of sickle cell anemia
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Diffuse Large b cell lymphoma
Low in CML

45. What are the labs and tx for HS?
Leukemia
Helminth infections major basic protein
Folate/B12 - nonmegaoblastic anemias - liver dz - EtOH (non folate/B12) reticulocytosis - metabolic disorder - drugs
Positive osmootic fragility test and splenectomy

46. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Degrades fibrin mesh and converts C3 to C3a
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Glanzmann's throbmasthenia
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

47. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Howell Jolly bodies
Folate def
Low O2 in papilla; can also get microhematuria from medullary infarcts
Helminth infections major basic protein

48. Is HS extravascular or intravascular?
Cromolyn sodium
Extravascular
SLE - CLL - alpha methyldopa
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

49. What is the therapy for the M3 variant?
Intravascular hemolysis
All trans retinoic acid
CML
Blood

50. What is appropriate absolute polycythemia
Both alpha and beta thal
Inc RBC - dec O2 sat - inc EPO
PGI2 - NO inc blood flow - dec platelet aggregation
B12/folate def