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Hemeonc

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Answer 50 questions in 15 minutes.
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1. What is a metabolic disorder tht can cause macrocytic anemia?
Lysine for glutamate at position 6
Orotic aciduria
Tissue
40 to 75

2. What is the therapy for CML (philly chrom)
Hereditary spherocytosis
Aspirin
Imatinib - anti bcr abl
No

3. Drug that inhbits the GpIIb/IIIa directly
Abciximab
Inc large vWF multimers - inc platelet aggregation and thrombosis
Cromolyn sodium
B antigena and A antibodes

4. What symptoms are associated with Hodgkin lymphoma
20 to 40
Fe def - thal - pb poison - sideroblastic anemia
Bite cells and Heinz bodies
B symptoms - fever night sweats - weight loss

5. Plasma cell neoplasm
Pyruvate kinase def - extravascular
Multiple myeloma
Dimercaprol and EDTA 1st line of treatment - succimer for kids - it sucks to be a kid who eats lead
Heinz bodies - seen in alpha thal and G6PD

6. What distinguishes multiple myeloma fromk Waldenstroms macroglobulinemia?
GpIIb/IIIa
No lytic bone lesions in WM
No platelet clumping
Inhibits thrombin - ixa - xa - xiia - and activated by heparin

7. What are the presenting symptoms of acute intermittent porphyria
Africa = Jaw lesion - US = pelvis or abdomen
Leukemia
Lymphoma
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -

8. What is the age breakdown for hodgkins
Fibrinogen
Blod - tissue - MACS
Bimodal - men>women - except for nodular sclerosing type
Gp1b

9. What is the Ddx for nonhemolytic normocytic anemia
Lysine for glutamate at position 6
Tissue
Varying sizes
Anemia of chronic disease - Aplastic anemia - kidney disease

10. What is makes a leukemia acute
Blasts > 5%
Factor V Leiden
Anemia of chronic disease - Aplastic anemia - kidney disease
Hairy cell leukemia

11. What causes the jaundice in extravascular hemolysis
Inc UCB
Up - down - up
Ticlopidine/clopidogrel
A antigen and B antibodies

12. What is contained within the azurophilic granules of PMNs
Peroxidase
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
HbC disease - Asplenia - Liver disease - thalassemia - 'HALT the hunter said to his target'
Fe def - thal - pb poison - sideroblastic anemia

13. What is the defect in beta thal?
Hereditary spherocytosis - autoimmune hemolysis
Splicing sites and promotor sequences
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
Low O2 in papilla; can also get microhematuria from medullary infarcts

14. What cell is primarily involved in non Hod lymph
AML
B cells
Blistering cutaneous photosens - most common porphyria
Dec synthesis of factors 1972 - protein C/S

15. Elderly - mature b cell tumor with filamentous - hairlike projections
Inc large vWF multimers - inc platelet aggregation and thrombosis
Burkitt lymphoma
Cold - seen in CLL - mycoplasma pneumoniae infections or infectious mononucleosis
Hairy cell leukemia

16. What virus is associated with Burkitt lymphoma
Helps platelts adhere to endothelium
EBV
Paraprotein spike - monoclonal protein
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils

17. Who has more severe disease - HbSS or HbSC
A antigen and B antibodies
HbSS
Multiple myeloma
Hereditary spherocytosis

18. Spherocyte
Hereditary spherocytosis - autoimmune hemolysis
Common a few days after oxidative stress in a pt with G6PD
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
...

19. What are the etiologies of B12 def
Malaria - Babesia
Megakaryocytes
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
CD15 and CD30 pos

20. What is the tx for sickle cell
Incactivates II - VII - IX - X - XI - XII
Hydroxyurea - bone marrow transplant
Complications of sickle cell anemia
Bleeding - malnutrition/absorption - inc demand pregs

21. Production of mutant factor V that cannot by degraded by protein C - most common cause of inherited hypercoaguability
Glucose and heme - which inhibit ALA synthase
Uroporphyrin (tea colored urine)
Factor V Leiden
Mycosis fundgoides/Sezary syndrome

22. Where do B cells arise from - mature - and migrate to...
TXA2 - dec blood flow - inc platelet aggregation
Extrinsic - I - II - V - VII and X
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Round densly staining nucleus with a small amount of pale cytoplasm

23. deficiency in ADAMTS13 leading to dec degradation of vWF
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
VIII
Thrombotic thrombocytopenic purpura
Africa = Jaw lesion - US = pelvis or abdomen

24. What do you see in vit K def
Porphobilinogen - delta ALA - uroporphyrin
Splicing sites and promotor sequences
Eleveated PT - PTT
Epoxide reductase - warfarin inhibits

25. What are the main associations with multiple myeloma?
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
Low in CML
SLE - CLL - alpha methyldopa
No antigen - both antibodies

26. hemolytic in a newborn - dec ATP and rigid RBCs
Lysine for glutamate at position 6
Birbeck granules
Liver disease - abetalipoproteinemia - acntho = spiny
Pyruvate kinase def - extravascular

27. What is the therapy for the M3 variant?
Orotic aciduria
All trans retinoic acid
Deficiency in factor VIII
Inc vasodiltion - inc perm - inc pain

28. What are the labs and tx for HS?
Positive osmootic fragility test and splenectomy
Von Willebrand's disease
Schistocytes and inc LDH
Bone marrow - thymus - blood (80% of circulating lymphos are T)

29. What does CRAB stand for in multiple myeloma
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
Antigen - antibody
Lacunar in nodular sclerosing variant
B12 def

30. How does the therapy for M3 vairant work?
Indirect coombs - agglutinate if serum anti RBC surface Ig
Lung disease - congenital heart diseaes - and high altitude
Hereditary spherocytosis
Induces differentiation of myeloblasts

31. What happens in beta thal major?
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Kids= exposure to lead paint - adults = battery - ammunition factory
Inc

32. What is the mutation in HbC
Extrinsic - I - II - V - VII and X
Indirect coombs - agglutinate if serum anti RBC surface Ig
Sickle cell anemia
Lysine for glutamate at position 6

33. Blood type B
Lead poisoning - rRNA causes aggregration of ribosomes and basophilic stippling
Blistering cutaneous photosens - most common porphyria
B antigena and A antibodes
Dec synthesis of factors 1972 - protein C/S

34. Which substrates begin the heme synthesis pathway
Fe def - thal - pb poison - sideroblastic anemia
B6 therapy (pyrodixine)
Glycine and succinyl - coa
EBV

35. What are some causes of warm agglutinin autoimmune hemolytic anemia?
SLE - CLL - alpha methyldopa
Degrades fibrin mesh and converts C3 to C3a
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia

36. What is the ddx for aplastic anemia

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37. Schisotcyte - helmet cell
DIC - TTP/HUS - traumatic hemolysis
IgG - Warm is GREAT
Intrinsic - all factors except - VII - XIII
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites

38. What is the pathogenesis of TTP?
Inc large vWF multimers - inc platelet aggregation and thrombosis
Blasts > 5%
HIV or immunosupression
Anemia of chronic disease - Aplastic anemia - kidney disease

39. What substance prevents mast cells degranulation?
Hereditary spherocytosis - autoimmune hemolysis
Cromolyn sodium
Varying sizes
Inc serum iron - normal TIBC - inc ferratin

40. Where are 1/3 of platelets stored
Howell Jolly bodies
Inc serum iron - normal TIBC - inc ferratin
Inc UCB
Spleen

41. Can B cells function as APCs?
Nl - up - nl
Multiple myeloma
Ringed sideronblasts with iron laden mitochondria
Yes - via MHC II

42. Where are basophils found?
Induces differentiation of myeloblasts
Blood
Inc
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)

43. What does LEAD stand for in lead poisoning?
Lead lines on gingivae and epiphyses of long bones on xray - Encephalopathy and Erythrocyte basophilic stippling - Abdominal colic and Anemia - Drops - wrist and foot drop -
Varying sizes
Prothrombin gene mutation
5- FU - AZT - hydroxyurea

44. What do you see in peripheral smear in a pt with G6PD?
Bite cells and Heinz bodies
Hb Barts - gamma4 in defect in all 4 alpha genes
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
Inc UCB

45. What is the treatment for acute intermittent porphyria
T(9;22) bcr abl
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Glucose and heme - which inhibit ALA synthase
Beta chain underproduced - asymptomatic - heterozygote

46. Where to T cells arise and mature - and migrate to...
Fe def - thal - pb poison - sideroblastic anemia
120 days
VWD
Bone marrow - thymus - blood (80% of circulating lymphos are T)

47. What activates the intrinsic pathway?
Low O2 in papilla; can also get microhematuria from medullary infarcts
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
Collagen - BM - activated platelets
Mycosis fundgoides/Sezary syndrome

48. Why does B12 def cause neuro sx?
CNS and testis
Signs and sx of aplastic anemia
Protein C or S deficiency
B12 in fatty acid pathways leads to subacute combined degeneration

49. What chromosomal translocation is associated with a better prognosis in All
Howell Jolly bodies
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
T(12;21)
Intravascular hemolysis

50. Which pathway and factorrs are tested by the PT coag test
Macrohemorrhage - hemarthroses - easy bruising
Factor V resistant to activated protein C's inhibition
Extrinsic - I - II - V - VII and X
...

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