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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. X linked - dec glutathione inc RBC susceptibility to oxidative stress
G6PD
Leukemoid reaction
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Paraprotein spike - monoclonal protein

2. What is the presenting scenario for TTP?
DIC
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Examples of oxidative stress
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT

3. What are the anti aggregation factors?
G6PD
PGI2 - NO inc blood flow - dec platelet aggregation
Produce antibody - lots of RER and golgi
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin

4. Elderly - mature b cell tumor with filamentous - hairlike projections
CML to AML or All
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Hairy cell leukemia
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease

5. Impaired synthesis of DAF leading to inc complement mediated desctruction of RBCs via the GPI ancho
Paroxysmal nocturnal hemoglobinuria
Inc lymphoctes - and less RS cells
Hereditary spherocytosis
TPA - cleavage of fibrin mesh

6. Sickle cell
Lung disease - congenital heart diseaes - and high altitude
Varying sizes
Inc HbF and dec HbS
Sickle cell anemia

7. S-100 and CD1a with birbeck granules
Langerhans cell histiocytosis
2 to 10
HIV or immunosupression
Aplastic anemia - pancytopenia

8. Where does All spread
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
CNS and testis
Extravascular hemolysis

9. normal RBCs added to patient's serum
Paraprotein spike - monoclonal protein
Indirect coombs - agglutinate if serum anti RBC surface Ig
Essential thrombocytosis
Gp1b

10. What are the pro aggregation factors?
Bite cells and Heinz bodies
TXA2 - dec blood flow - inc platelet aggregation
Radiation/drugs - viral agents - fanconi's anemia - idiopathic may follow acute hepatitis
Decrease EPO

11. What is hemophiliia A
Dec synthesis of factors 1972 - protein C/S
Deficiency in factor VIII
Polycythemia vera
Anemia of chronic disease - Aplastic anemia - kidney disease

12. What is the most common hodgkin lymphoma
Inc UCB
Nodular sclerosing
Parvovirus
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

13. Aplastic crisis after parvovirus - autosplenectomy - salmonella osteomyelitis - painful crisis - renal papillary necrosis - splenic sequestration
IgG - Warm is GREAT
Adult T cell lymphoma - presents with cutaneous lesions
Malaria - Babesia
Complications of sickle cell anemia

14. What is the clinical picture of hemophilia A or B
Hodgkin
Macrohemorrhage - hemarthroses - easy bruising
Dec synthesis of factors 1972 - protein C/S
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)

15. What vitamin is a cofactor for the first step of heme synthesis
Induces differentiation of myeloblasts
Glanzmann's throbmasthenia
Malaria - Babesia
B6

16. What is appropriate absolyte polycythemia associated with
G6PD
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Lung disease - congenital heart diseaes - and high altitude
They bind vWF via GpIb

17. What are the age ranges for the various leukemias
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
No
MHC II and Fc receptors
TXA2 - dec blood flow - inc platelet aggregation

18. Back pain - hemoglobinuria
HIV or immunosupression
Ewing sarcoma
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Common a few days after oxidative stress in a pt with G6PD

19. What causes renal papillary necrosis in sickle cell
Low O2 in papilla; can also get microhematuria from medullary infarcts
IFN gama
Beta 4
Malaria - Babesia

20. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
ATIII def
Lead - and EtOH
Low in CML
PGI2 - NO inc blood flow - dec platelet aggregation

21. Where do you see hypersegmented polys?
B12/folate def
Low O2 in papilla; can also get microhematuria from medullary infarcts
Bite cells and Heinz bodies
Inc RBC - dec O2 sat - inc EPO

22. What is the accumulated substance in lead poisoning
Round densly staining nucleus with a small amount of pale cytoplasm
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Protoporphyrin (blood)

23. hypersegmented PMNs - glossitis - dec folate - inc homocysteine - nl methylmalonic acid
Sickle cell
No lytic bone lesions in WM
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Folate def

24. iron deficiency anemia - esophageal web - atrophic glossitis
40 to 75
Inc vasodiltion - inc perm - inc pain
Orotic aciduria
Plummer - vinson syndrome

25. What is the treatment for lead poising?
Bone marrow - thymus - blood (80% of circulating lymphos are T)
B symptoms - fever night sweats - weight loss
Hydroxyurea - bone marrow transplant
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead

26. What does decreasing heme do to ALA synthase activity?
Epoxide reductase - warfarin inhibits
Inc
Leukemoid reaction
Inducers of primary antibody response

27. t(11:22)
Fc
Ewing sarcoma
Aspirin
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination

28. What does STOP Making New Thrombi stand for
Valine for glutamate
G6PD
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
20 to 40

29. What reveresible things can a sideroblastic anemia
Lead - and EtOH
Varying sizes
Both alpha and beta thal
Plummer - vinson syndrome

30. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
No lytic bone lesions in WM
Schistocytes and inc LDH
Nl - up - nl
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

31. What do you see in vit K def
Pyruvate kinase def - extravascular
Bleeding - malnutrition/absorption - inc demand pregs
Eleveated PT - PTT
Sideroblastic anemia

32. What are the presenting symptoms of acute intermittent porphyria
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Protein C or S deficiency
B6 therapy (pyrodixine)
Imatinib - anti bcr abl

33. What is the philadelphia chromosome
T(9;22) bcr abl
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Thrombotic thrombocytopenic purpura
Sideroblastic anemia

34. What are the etiologies of B12 def
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
No antigen - both antibodies
TRAP (tartrate resistant acid phosphatase
Peripheral neuropathy - posterior columns (vibration/proprioception) - lateral corticospinal (spasticity) - dementia

35. Serum iron - transferrin - ferritin lab values for anemia of chronic disease
Down - down - up
Easy gas exchange
Collagen - BM - activated platelets
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT

36. Bone pain plus anemia
Acts to stabilize platelet plug
Def in factor IX
Exposed collagen upon endothelial damage
Multiple myeloma

37. Eosinophils are highly phagocytic For what kind of complex?
SLE - CLL - alpha methyldopa
Peroxidase
Antigen - antibody
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT

38. What activates the fibrinolytic pathway?
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
B6
XIIa activates kallikrein - which activates plasmin (and converts HMWK to bradykinin
No

39. What substance prevents mast cells degranulation?
Malaria - Babesia
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Cromolyn sodium
Up - down - nl

40. What does bradykinin do?
Both alpha and beta thal
Inc vasodiltion - inc perm - inc pain
Induces differentiation of myeloblasts
Anemia of chronic disease - Aplastic anemia - kidney disease

41. poikilocytosis
Varying shapes
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Gp1b

42. What is the characteristic spread of Hodgkin Lymphoma
Contiguous
Nl PT - elevated PTT - intrinsic pathway defect
Uroporphyrinogen decarboxylase
Deficiency in factor VIII

43. What is the purpose of the fibrin mesh
Positive osmootic fragility test and splenectomy
Multiple myeloma
Factor V Leiden
Acts to stabilize platelet plug

44. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
90% anearobically from glucose to lactate - 10% from HMP shunt
Down - down - up
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
Off center nuclues - clock face chromatin

45. How does the therapy for M3 vairant work?
40 to 75
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Dec
Induces differentiation of myeloblasts

46. Spherocyte
Hereditary spherocytosis - autoimmune hemolysis
Ticlopidine/clopidogrel
M3 AML (acute promyelocytic leukemia)
Helminth infections major basic protein

47. What is the tx for sickle cell
Hereditary spherocytosis
2 to 10
Hydroxyurea - bone marrow transplant
Dec

48. What is the coombs test results in AIHA?
Tissue
Pos
Megaloblastic anemia (also hypersegmented PMNs - marrow failure)
Produce antibody - lots of RER and golgi

49. Adults present with cutaneous patches/nodules - indolent CD4+
B12 in fatty acid pathways leads to subacute combined degeneration
Mycosis fundgoides/Sezary syndrome
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

50. What is the general pathology of a macrocytic anemia?
TXA2 - dec blood flow - inc platelet aggregation
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Blistering cutaneous photosens - most common porphyria
Hereditary spherocytosis - G6PD - sickle cell