mjl1717
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initial test--factor VIII and immunoreactive vWF llevels
confirm with---Ristocetin cofactor assay
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| DrVirgo
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Right.
WHY are factor VIII and vWF NOT decreased in liver disease in which all the other factors are decreased?
And how do you do the mixing studies?
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| DrVirgo
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vWF and Factor VIII are made in endothelial cells whereas the other factors are made in the liver so in liver disease vWF and VIII don't decrease.
Can anyone explain how the mixing studies are done?
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| chemamr
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how is this Von Willebrand Dz treated?
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| mjl1717
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DDAVP/ or FFP
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| DrVirgo
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DDAVP in MILD bleeding or pre-op.
-It releases subendothelial stores of vWF.
Better treatment is the actual vWF factor replacement.
FFP is NOT useful.
Cryoprecipitate (which contains vWF as one of its components) WAS used in the past but now that we have vWF factor replacement, we just give that.
--To diagnose vWD, do the Ristocetin Platelet aggregation test which measures the ability of platelets to bind to an artificial endothelial surface (ristocetin).
Ristocetin is mixed with platelets.
If aggregation occurs, this means that vWF IS present, and working. Patient is normal.
If there is NO aggregation, this means there is no vWF and patient has vWD.
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| mjl1717
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Good Dr Virgo- good looking out.. I guess it would have been better if Id said Factor VIII concentrate that contains specific vWf replacement for severe disease. (Rx changes real fast)
w
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