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25 Cards in this Set
- Front
- Back
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what is the source of vWF
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Weibel-palade bodies of endothelial cells and alpha granules of platelets
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what is the MOA of ticlopidine and copidogrel
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inhibit the ADP-induced expression of GpIIb/IIIa
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what is the MOA of abciximab
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inhibit GpIIb/IIIa
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how can you Rx a pt with von willebrand disease?
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givem desmopressin which causes a release of vWF from weibel palade bodies
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what will the free erythrocyte protoporphyrin (FEP) levels be with iron deficiency anemia?
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High
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where does the final step of heme synthesis occur?
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in the mitochondria under the control of ferrochelatase
thats why you see iron laden mitochondria in sideroblastic anemia (iron isnt joining up with the defetive protoporphyrin and just sits there in the mitochondria) |
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cis and trans deletions of the alpha globin deletions: which occurs in asians and which occurs in africans
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CATA
Cis----Asians Trans---Africans |
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what chromosome is the alpha-globin gene on
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16
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HbH hemoglobin is seen in deletion of how many genes in alpha thalassemia
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3
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what kind of cells are seen in beta thalassemia
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target cells
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skull xray in beta thalassemia shows what
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"crew cut" due to expansion of hematopoiesis
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methylmalonic acid will be high in what deficiency causing macrocytic anemia?
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vitamin B12
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which takes years to develop....vitamin B12 or folate deficiency?
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vitamin B12
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what is the aa substitution in sickle cell anemia?
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valine instead of glutamic acid at position 6
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what does hydroxyurea do for a sickle cell patient?
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increases HbF which is resistant to sickling
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what is a common presentation in infants with sickle cell anemia?
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dactylitis (swollen hands and feet) from vaso-occlusive infarcts in bones
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what is the cause of PNH?? (other than the DAF deficiency)
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defect in myeloid stem cell resutling in absence of GPI (which secures DAF to cell surfaces)
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10% of pt with PNH can develop this?
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acute myeloid leukemia
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inheritance pattern of G6PD
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X linked
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what is the most common inherited bleeding disorder and what is the inheritance pattern
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von willebrands disease
autosomal dominant |
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most common type of hodgkins lymphoma? prognosis?
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nodular sclerosing
good prognosis |
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"starry-sky" appearance of cells = ??
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burkitts lymphoma
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hairy cell leukemia responds well to what Rx
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2-CDA (cladribine)
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patient has red wine colored urine, painful abdomen, polyneuropathy.....what they got?
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acute intermittent porphyria
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patient has porphobilinogen deaminase enzyme inhibited....what they got?
treatment? |
acute intermittent porphyria
rx with glucose and heme |
