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Hemeonc
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Subject
:
health-sciences
Instructions:
Answer 50 questions in 15 minutes.
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Match each statement with the correct term.
Don't refresh. All questions and answers are randomly picked and ordered every time you load a test.
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 level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Low in CML
Intrinsic - all factors except - VII - XIII
Howell - Jolly bodies - hypo/asplenia
Varying sizes
2. Defect in platelet to collagen adhesion - receptor - disorder - platelet count and BT
Blasts > 5%
Plummer - vinson syndrome
Leukemoid reaction
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
3. What do labs show in DIC?
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
Dec platelet survival and inc megakaryocytes - thrombocytopenia and inc BT
Sideroblastic anemia
Schistocytes - inc fibrin split products (D- dimers) - dec fibrinogen - dec factors V and VIII
4. inc LDH - jaundice
Mantle cell lymphoma
Diffuse Large b cell lymphoma
Extravascular hemolysis
Rhogam - Rh antigen immunoglobulin
5. What finding you do you see in patients after splenectomy
Howell Jolly bodies
Schistocytes and inc LDH
VWD
Inc large vWF multimers - inc platelet aggregation and thrombosis
6. What is contained within the azurophilic granules of PMNs
Dec synthesis of factors 1972 - protein C/S
Fibrinogen
Cromolyn sodium
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
7. What is the ddx for aplastic anemia
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8. What are the etiologies of folate def
Lysine for glutamate at position 6
Neoplastic - asthma - allergic processes - collagen vacsular disease - parasites
Inappropriate absolute with inc RBCs and EPO
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
9. Drug that inhbits the GpIIb/IIIa directly
B6 therapy (pyrodixine)
Plasma cell
Abciximab
Varying sizes
10. What is the difference of presentation of Burkitt in Africa vs the United States
Africa = Jaw lesion - US = pelvis or abdomen
Off center nuclues - clock face chromatin
Pyruvate kinase def - extravascular
HyperCalcemia - Renal insuff - Anemia - Bone lytic lesions/Back pain
11. can be asymptomatic - often in older adults - peripheral blood lymphcytosis with smudge cells and warm antibody autoimmune hemolytic anemia
Low in CML
Low O2 in papilla; can also get microhematuria from medullary infarcts
CLL (SLL without the peripheral lymphocytosis
5- FU - AZT - hydroxyurea
12. RBCs damagaed from passing through obstructed - narrowed vessel lumina
Common a few days after oxidative stress in a pt with G6PD
MAHA
Protoporphyrin
Bone marrow - thymus - blood (80% of circulating lymphos are T)
13. What cell is primarily involved in non Hod lymph
B cells
Deficiency in factor VIII
Yes - via MHC II
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
14. Oxidation of iron - denatured hemoglobin preceipitation damage to RBC membrane - formation of bite cells
40 to 50
Heinz bodies - seen in alpha thal and G6PD
Schistocytes and inc LDH
Langerhans cells
15. What is appropriate absolute polycythemia
Schistocytes and inc LDH
Intrinsic - all factors except - VII - XIII
Inc RBC - dec O2 sat - inc EPO
SLE - CLL - alpha methyldopa
16. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
Epoxide reductase - warfarin inhibits
Intravascular hemolysis
IFN gama
17. mother's antibodies attack fetal RBCs
Factor V resistant to activated protein C's inhibition
Abciximab
Mycosis fundgoides/Sezary syndrome
Erythroblastosis fetalis - Rh or other blood antigen incompatibility
18. What do auer rods stain with
EBV
Sickle cell anemia
Lysine for glutamate at position 6
Peroxidase
19. What activates protein C and What does activated protein C do?
G6PD
Protein S - cleaves and invactivates Va and VIIIa
Pos
Round densly staining nucleus with a small amount of pale cytoplasm
20. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Easy gas exchange
AB - no antibodies
Incactivates II - VII - IX - X - XI - XII
Sideroblastic anemia
21. What is the pattern of involvement and spread for nonHod lympho
Cromolyn sodium
Multiple - peripheral nodes - extranodal involvement - noncontiguous spread
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
TRAP (tartrate resistant acid phosphatase
22. What is the tx for sickle cell
Up - down - nl
Hydroxyurea - bone marrow transplant
Schistocytes and inc LDH
Factor V Leiden
23. What is the mutation in HbS
All trans retinoic acid
TRAP (tartrate resistant acid phosphatase
Valine for glutamate
AML
24. poikilocytosis
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Exposed collagen upon endothelial damage
Varying shapes
Inc HbA2 on electrophoresis
25. What is the main source of energy in RBCs
Tissue factor converst the VII and VIIa
90% anearobically from glucose to lactate - 10% from HMP shunt
Malnutrition - malabsoprtion - impaired metabolism - inc requirement as in an hemolytic anemia or pregnancy
2 to 10
26. Eosinophils are highly phagocytic For what kind of complex?
Dec
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
B12 in fatty acid pathways leads to subacute combined degeneration
Antigen - antibody
27. What is monoclonal expansion without symptoms associated with multiple myeloma?
Beta thal
Folate def
Lysine for glutamate at position 6
MGUS - monoclonal gammopathy of undetermined significance
28. When do you see MAHA?
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
Acts to stabilize platelet plug
ADP and Ca
29. Sickle cell
Leukemia
Lung disease - congenital heart diseaes - and high altitude
TPA - cleavage of fibrin mesh
Sickle cell anemia
30. Defect in proteins interacting with RBC membrane skeleton and plasma membrane
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 -
Glanzmann's throbmasthenia - GpIIb/IIIa - nl platelets - inc BT
Indirect coombs - agglutinate if serum anti RBC surface Ig
Hereditary spherocytosis
31. What is the life spance of a platelet?
Induces differentiation of myeloblasts
8-10 days
Langerhans cell histiocytosis
Factor V Leiden
32. What substance prevents mast cells degranulation?
Protein S - cleaves and invactivates Va and VIIIa
Cromolyn sodium
Direct coombs - agglutinate if RBCs are coated with Ig
Inc lymphoctes - and less RS cells
33. philadelphia chromosome - blood looks like marrow
Reed - sternberg cells
ATIII def
Down - down - up
CML
34. Which drugs can cause macrocytic anemia?
5- FU - AZT - hydroxyurea
Blood
Megakaryocytes
All trans retinoic acid
35. What is the activation stage of platelet plug formation?
Up - down - up
Leukemia
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
Protein S - cleaves and invactivates Va and VIIIa
36. What causes renal papillary necrosis in sickle cell
Lung disease - congenital heart diseaes - and high altitude
IFN gama
Lysine for glutamate at position 6
Low O2 in papilla; can also get microhematuria from medullary infarcts
37. Deficiency in vWF
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38. What vitamin is a cofactor for the first step of heme synthesis
Thrombotic thrombocytopenic purpura
B6
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Hereditary elloptocytosis
39. Where are 1/3 of platelets stored
Eleveated PT - PTT
40 to 75
Spleen
Uroporphyrinogen decarboxylase
40. Does CML have a JAK2 mut
Paroxysmal nocturnal hemoglobinuria
Signs and sx of aplastic anemia
CML
No
41. bcl -2 t(14;18) adults
Inc UCB
Down - up - down
Follicular lymphoma - indolent course
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
42. What is the treatment for acute intermittent porphyria
Eleveated PT - PTT
Glucose and heme - which inhibit ALA synthase
Beta thal
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
43. What is the tx for vWD
Langerhans cells
HIV or immunosupression
Eleveated PT - PTT
DDAVP (desmopressin) which releases stored vWF stored in endothelium
44. What is makes a leukemia acute
Tissue factor converst the VII and VIIa
Blasts > 5%
Rhogam - Rh antigen immunoglobulin
B12 in fatty acid pathways leads to subacute combined degeneration
45. Where do B cells arise from - mature - and migrate to...
Positive osmootic fragility test and splenectomy
TPA - cleavage of fibrin mesh
Hb Barts - gamma4 in defect in all 4 alpha genes
Marrow - marrow - follicles of lymph nodes - white pulp of spleen (unencapsulated lymphoid tissue)
46. t(11;14)
GpIIb/IIIa
Petechiae
90% anearobically from glucose to lactate - 10% from HMP shunt
Mantle cell lymphoma
47. Why can newborns with sickle cell be asymptomatic
Inc HbF and dec HbS
Polycythemia vera with inc plasma volume - inc RBCs - inc EPO
Leukemia
EBV
48. What converts plasminogen to plasm and What does plasmin do?
TPA - cleavage of fibrin mesh
Birbeck granules
Inc UCB
Dec
49. What are the presenting symptoms of porphyria cutanea tarda
Inc
VIII
Blistering cutaneous photosens - most common porphyria
Lung disease - congenital heart diseaes - and high altitude
50. Back pain - hemoglobinuria
Chloride bicarb antiport system - - allows transport of C02 from periph to lungs
Intravascular hemolysis
Nl PT - elevated PTT - intrinsic pathway defect
Common a few days after oxidative stress in a pt with G6PD
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