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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. X linked defect in delta aminolevulinic acid synthase gene causes a defect in hemesynthesis
Sideroblastic anemia
Ewing sarcoma
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
ADP binding to receptors causes upregulation of GpIIb/IIIa expression at platelet surface
2. What percentage of WBCs are eosinophils?
1 to 6
Inc RBC - dec O2 sat - inc EPO
90% anearobically from glucose to lactate - 10% from HMP shunt
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
3. What begins the extrinsic pathway?
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Tissue factor converst the VII and VIIa
Lymphoma
Dec
4. What is the Ddx for a normocytic - normochromic anemia?
Extravascular
Hemolytic or non hemolytic - and then for the hemolytiuc - intravascular hemolysis or extravascular hemolysis
Beta thal
Low in CML
5. What level of leukocyte alkaline phosphatase is expected in CML versus leukemoid reaction
Sickle cell anemia
Low in CML
Yes - via MHC II
Histaminase and arylsulfatase
6. What the alpha granules contain in platelets?
IgG - Warm is GREAT
VWF and fibrinogen
B6 therapy (pyrodixine)
Africa = Jaw lesion - US = pelvis or abdomen
7. What is relative polycythemia
IFN gama
Inflammation leads to inc hepcidin causing a dec in release from iron from macs: dec serum fe - dec TIBC - inc ferritin
Dec plasma volume
...
8. What vitamin is a cofactor for the first step of heme synthesis
Bernard soulier
All
Low in CML
B6
9. Which infections can cause MAHA?
No
Malaria - Babesia
Allogenic bone marrow transplant - RBC/platelet transfusion - C- GSF - or GM- CSF
VWF carries/protects factor VIII
10. Serum iron - transferrin - ferritin lab values for lead poisoning anemia
Kids= exposure to lead paint - adults = battery - ammunition factory
Acts to stabilize platelet plug
Up - down - nl
Insufficient intake as with strict vegans - malabsorption as in crohns - pernicious anemia - diphyllobothrium
11. universal donor
Eleveated PT - PTT
No antigen - both antibodies
Inc serum iron - normal TIBC - inc ferratin
All < 15 - AML = median onset around 60 - CML = 30 to 60 - and CLL > 60
12. In a Rh - mother who has developed anti Rh antibodies - who is at risk of hemolytic disease?
Her next Rh+ fetus
Abciximab
Hydrolytic enzymes - lysozyme - myeloperoxidase - lactoferrin
Myelofibrosis (marow is crying being its fibrosed'
13. Intrinsic pathway coagulation defect and defect in platelet plug formation - most common inherited bleeding disorder - AD
Helps platelts adhere to endothelium
Schistocytes - helmet cells
VWD
40 to 50
14. universal recipient
AB - no antibodies
Aplastic anemia - pancytopenia
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
VWD
15. What are some causes of warm agglutinin autoimmune hemolytic anemia?
Leukemia
Produce antibody - lots of RER and golgi
Dendritic cells?
SLE - CLL - alpha methyldopa
16. What does antithrombin do and What activates it?
Protein S - cleaves and invactivates Va and VIIIa
Gp1b - bernard - soulier - dec platelets (giant platelets not counted) - inc BT
B12 def
Inhibits thrombin - ixa - xa - xiia - and activated by heparin
17. What is the pathogenesis of TTP?
Inc large vWF multimers - inc platelet aggregation and thrombosis
Porphobilinogen - delta ALA - uroporphyrin
< 1% - heparin - histamine - LTD-4 - other vasoactive amines
Up - down - up
18. What virus can cause an aplastic crisis in pts with HS?
Parvovirus
Folate def
CML
B6
19. What is the characteristic histologic finding in Hodgkin Lymphoma
Reed - sternberg cells
Painful abdomen (also in lead poisoning) - red wine colored urine - polyneuropathy - psychological distrubances - precipated by drugs -
Eleveated PT - PTT
Helps platelts adhere to endothelium
20. What is the characteristic lab finding on electrophoresis
Paraprotein spike - monoclonal protein
All trans retinoic acid
Contiguous
They bind vWF via GpIb
21. What are the two substances produced by eosinophils help limit the reaction following mast cell degranulation?
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
Defect in 4 - not compatible with life - defect in 3 - HbH disease - defect in 1/2 not associated with sig anemia
Schistocytes - helmet cells
Histaminase and arylsulfatase
22. What is the treatment to prevent a woman from forming anti Rh antibody?
B symptoms - fever night sweats - weight loss
VWF and fibrinogen
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Rhogam - Rh antigen immunoglobulin
23. After an injury - What does vWF bind to begin platelet plug formation?
EBV
Protoporphyrin
Exposed collagen upon endothelial damage
Factor V resistant to activated protein C's inhibition
24. What is makes a leukemia acute
Blasts > 5%
Fc
Up - down - nl
90% anearobically from glucose to lactate - 10% from HMP shunt
25. What does the blood smear show in glanzmann's?
No platelet clumping
Inactivates it
Round densly staining nucleus with a small amount of pale cytoplasm
Essential thrombocytosis
26. What do you see in peripheral smear in a pt with G6PD?
Hb Barts - gamma4 in defect in all 4 alpha genes
Bite cells and Heinz bodies
Orotic aciduria
Intravascular
27. When is the peak incidence for nonHod lymphoma
20 to 40
Inc HbA2 on electrophoresis
SLE - CLL - alpha methyldopa
Inc RBC - dec O2 sat - inc EPO
28. What do platelets interact with to form a hemostatic plug
Histamine - heparin - and eosinohil chemotactic factors
Rhogam - Rh antigen immunoglobulin
Fibrinogen
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
29. What do you see in vit K def
DIC
Eleveated PT - PTT
Dec synthesis of factors 1972 - protein C/S
TAIL - thalassemias - anemia of chronic disease - iron def - lead poisoning
30. philadelphia chromosome - blood looks like marrow
Myelofibrosis (marow is crying being its fibrosed'
Porphobilinogen - delta ALA - uroporphyrin
CML
Lead - and EtOH
31. Ddx for microcytic anemia
WBC diff highest to lowest: neutrophils - lymphocytes - monocytes - eosinophils - basophils
Leukemia
Imatinib - anti bcr abl
Fe def - thal - pb poison - sideroblastic anemia
32. What do eosinophils defend against and What do they use to do it?
B antigena and A antibodes
Helminth infections major basic protein
CML
PENTAD: neurologic sx - renal sx - fever - thrombocytopenia - MAHA
33. What does Vit K deficiency cause?
Helps platelts adhere to endothelium
Nodular sclerosing
Liver disease - abetalipoproteinemia - acntho = spiny
Dec synthesis of factors 1972 - protein C/S
34. When do you see MAHA?
Ankryin - band 4.1 or spectrin
Paraprotein spike - monoclonal protein
Porphobilinogen - delta ALA - uroporphyrin
DIC - TTP- HUS - SLE malignant HTN - prostethic heart valves - aortic stenosis
35. normal RBCs added to patient's serum
Inc RBC - dec O2 sat - inc EPO
Protein C or S deficiency
MHC II and Fc receptors
Indirect coombs - agglutinate if serum anti RBC surface Ig
36. What activates protein C and What does activated protein C do?
Blod - tissue - MACS
CNS and testis
DNA synth impaired - maturation of nucleus delayed relative to maturation of cytoplasm
Protein S - cleaves and invactivates Va and VIIIa
37. How is beta thal minor dx
Up - down - nl
Inc suscept to infxn - primary amyloidosis - punched out lytic bone lesions - M spike - Ig light chains in urine - Rouleaud formations (stacked RBCs)
DIC
Inc HbA2 on electrophoresis
38. Does CML have a JAK2 mut
No
Glanzmann's throbmasthenia
Extravascular hemolysis
Imatinib - anti bcr abl
39. adults - auer rods - inc circulating myeblasts on peripheral smear
DIC
Parvovirus
Sheets of lymphocytes interspersed with macs
AML
40. What is the affected enzyme in lead poisoning
Factor V resistant to activated protein C's inhibition
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
Ferrocheletase and ALA dehydrogenase
Direct coombs - agglutinate if RBCs are coated with Ig
41. Serum iron - transferrin - ferritin lab values for iron def anemia
M3 AML (acute promyelocytic leukemia)
Low O2 in papilla; can also get microhematuria from medullary infarcts
Langerhans cell histiocytosis
Down - up - down
42. What is the clinical picture of hemophilia A or B
Macrohemorrhage - hemarthroses - easy bruising
Folate def
Protein C or S deficiency
Intrinsic - all factors except - VII - XIII
43. What are the azuraphilic granules in PMNs
TXA2 - dec blood flow - inc platelet aggregation
Lysosomes
B cells
Lead - and EtOH
44. Widespread activation of clotting to a deficiency in clotting factors which causes a bleeding states
Kids= exposure to lead paint - adults = battery - ammunition factory
Inappropriate absolute with inc RBCs and EPO
Inc serum iron - normal TIBC - inc ferratin
DIC
45. What is the are the presenting symptoms of lead poisoning in kids and adults
Easy gas exchange
Beta chain absent - homozgote - severe anemia requiring blood transfusion - secondary hemochromatosis
TXA2 - dec blood flow - inc platelet aggregation
Kidney - Gi - microcytic anemai in both - kids = mental retardation and adults = headache - memory loss - demyelination
46. t(11;14)
Mantle cell lymphoma
20 to 40
Sheets of lymphocytes interspersed with macs
Up - down - up
47. What does bradykinin do?
Inc vasodiltion - inc perm - inc pain
Yes - via MHC II
Inc large vWF multimers - inc platelet aggregation and thrombosis
Varying shapes
48. What is the pathogenesis of sickle cell
GpIIb/IIIa
Lacunar in nodular sclerosing variant
Intrinsic - all factors except - VII - XIII
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
49. What does STOP Making New Thrombi stand for
Inc bleeding time - microhemorrhage: mucuous membrane bleeding - epitaxis - petechiae - purpura - possible thrombocytopenia
Deoxygenated HbS polymerizes - low O2 or dehydration precipiates sickling - anemia and veno - occlusive disease
Causes of DIC: Sepsis (gram neg) - Trauma - Obstetric complications - acute Pancreatitis - Malignancy - Nephrotic syndrome - Tranfusion
Lead - and EtOH
50. What does increasing heme do to ALA synthase activity
Acts to stabilize platelet plug
Dec
Beta thal
Spleen
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