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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. What are the azuraphilic granules in PMNs
Liver disease - abetalipoproteinemia - acntho = spiny
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Cromolyn sodium
Lysosomes

2. What does STOP Making New Thrombi stand for
Howell - Jolly bodies - hypo/asplenia
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Down - up - down

3. What converts plasminogen to plasm and What does plasmin do?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
TPA - cleavage of fibrin mesh
B12/folate def
Uroporphyrin (tea colored urine)

4. What substance is Fe added to to yield heme
Protoporphyrin
Rhogam - Rh antigen immunoglobulin
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
DIC

5. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Intrinsic - all factors except - VII - XIII
Up - down - nl
Factor V Leiden

6. anisocytosis
Glycine and succinyl - coa
CD15 and CD30 pos
Howell Jolly bodies
Varying sizes

7. What does bradykinin do?
Mantle cell lymphoma
Beta chain underproduced - asymptomatic - heterozygote
Africa = Jaw lesion - US = pelvis or abdomen
Inc vasodiltion - inc perm - inc pain

8. In hemophilia A or B What do you see in the coag tests
Histamine - heparin - and eosinohil chemotactic factors
Nl PT - elevated PTT - intrinsic pathway defect
Blood
8-10 days

9. What is the effect of ACE on bradykinin
MHC II and Fc receptors
Inactivates it
Multiple myeloma
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis

10. What happens in beta thal major?
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
All
Multiple myeloma
Factor V resistant to activated protein C's inhibition

11. What CD molecules are on RS cells
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
No
CD15 and CD30 pos

12. Who has more severe disease - HbSS or HbSC
Complications of sickle cell anemia
Leukemoid reaction
Leukemia
HbSS

13. Teardrop cell
DIC
Bone marrow infiltration - myelofibrosis
Diffuse Large b cell lymphoma
Inc HbA2 on electrophoresis

14. Wilm's tumor - RCC - HCC - hydronephrosis
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Her next Rh+ fetus
Intrinsic - all factors except - VII - XIII

15. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
CLL (SLL without the peripheral lymphocytosis
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Inc large vWF multimers - inc platelet aggregation and thrombosis

16. What do plasma cells do
Produce antibody - lots of RER and golgi
Induces differentiation of myeloblasts
MAHA
Paraprotein spike - monoclonal protein

17. Can B cells function as APCs?
Yes - via MHC II
Collagen - BM - activated platelets
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Langerhans cells

18. Inherited deficiency of antithrombin; reduced inc in PTT after administration of heparin
ATIII def
DIC - TTP/HUS - traumatic hemolysis
Malaria - Babesia
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs

19. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Signs and sx of aplastic anemia
SLE - CLL - alpha methyldopa
Down - up - down
Beta thal

20. Conditions of defective heme synthesis leading to accumulation of heme precurors
40 to 75
B symptoms - fever night sweats - weight loss
Porphyrias
Prothrombin gene mutation

21. What is the tx for aplastic anemia
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Glycine and succinyl - coa
2 to 10
Epoxide reductase - warfarin inhibits

22. What is the main source of energy in RBCs
Extrinsic - I - II - V - VII and X
Inc HbA2 on electrophoresis
...
90% anearobically from glucose to lactate - 10% from HMP shunt

23. What are the neuro sx of B12 def?
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Extrinsic - I - II - V - VII and X
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia
Multiple myeloma

24. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
Varying sizes
Idiopathic thrombocytopenia (ITP)
Sickle cell

25. Which infections can cause MAHA?
Mycosis fundgoides/Sezary syndrome
Malaria - Babesia
Varying shapes
Alpha thal - asian and african american

26. What are the main associations with multiple myeloma?
Tissue
Hodgkin
Beta thal
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

27. What causes hydrops fetalis
Lung disease - congenital heart diseaes - and high altitude
Hb Barts - gamma4 in defect in all 4 alpha genes
Burkitt lymphoma
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

28. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
No lytic bone lesions in WM
Sheets of lymphocytes interspersed with macs
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Helminth infections major basic protein

29. Blood type A
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
A antigen and B antibodies
Hairy cell leukemia
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

30. What is the receptor for fibrinogen?
T(9;22) bcr abl
Hereditary elloptocytosis
Howell Jolly bodies
GpIIb/IIIa

31. What is the affected enzyme in acute intermittment porphyria
Inc
Porphobilinogen - delta ALA - uroporphyrin
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
B antigena and A antibodes

32. What does ectopic EPO produce
Up - down - nl
Indirect coombs - agglutinate if serum anti RBC surface Ig
Inappropriate absolute with inc RBCs and EPO
B6

33. Elliptocyte
1 to 6
40 to 75
Paraprotein spike - monoclonal protein
Hereditary elloptocytosis

34. What does the blood smear show in glanzmann's?
GpIIb/IIIa
No platelet clumping
Induces differentiation of myeloblasts
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis

35. Where are 1/3 of platelets stored
Spleen
Sideroblastic anemia
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Lysine for glutamate at position 6

36. What is the characteristic lab finding on electrophoresis
Anti Rh
Paraprotein spike - monoclonal protein
Spleen
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin

37. hemolytic in a newborn - dec ATP and rigid RBCs
Lysosomes
Yes - via MHC II
Pyruvate kinase def - extravascular
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA

38. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Blod - tissue - MACS
Ringed sideronblasts with iron laden mitochondria
Sideroblastic anemia
Produce antibody - lots of RER and golgi

39. What signal activates MACS
Bone marrow - thymus - blood (80% of circulating lymphos are T)
IFN gama
Tissue
Bernard soulier

40. What is factor V leidin?

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41. What is appropriate absolyte polycythemia associated with
Sheets of lymphocytes interspersed with macs
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Inc UCB
Lung disease - congenital heart diseaes - and high altitude

42. bcl -2 t(14;18) adults
Follicular lymphoma - indolent course
Both alpha and beta thal
Mantle cell lymphoma
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling

43. What do the labs show for TTP?
Prothrombin gene mutation
Fc
Schistocytes and inc LDH
Pyruvate kinase def - extravascular

44. Which substrates begin the heme synthesis pathway
T(9;22) bcr abl
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Glycine and succinyl - coa
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis

45. normal RBCs added to patient's serum
Indirect coombs - agglutinate if serum anti RBC surface Ig
Complications of sickle cell anemia
Porphobilinogen - delta ALA - uroporphyrin
CNS and testis

46. What do they express on their surface?
40 to 50
MHC II and Fc receptors
ATIII def
Bimodal - men>women - except for nodular sclerosing type

47. Ddx for microcytic anemia
Fe def - thal - pb poison - sideroblastic anemia
Extrinsic - I - II - V - VII and X
Parvovirus
Rhogam - Rh antigen immunoglobulin

48. What is the purpose of the fibrin mesh
Uroporphyrin (tea colored urine)
Macrohemorrhage - hemarthroses - easy bruising
Kids= exposure to lead paint - adults = battery - ammunition factory
Acts to stabilize platelet plug

49. Which drugs can cause macrocytic anemia?
Glycine and succinyl - coa
5- FU - AZT - hydroxyurea
TPA - cleavage of fibrin mesh
B cells

50. What causes renal papillary necrosis in sickle cell
Low O2 in papilla; can also get microhematuria from medullary infarcts
Her next Rh+ fetus
Ticlopidine/clopidogrel
40 to 50