Article
Assay of the von Willebrand factor (VWF) propeptide to identify patients with type 1 von Willebrand disease with decreased VWF survival.
Blood - 15 Nov 2006
Haberichter Sandra L, Balistreri Michael, Christopherson Pamela, Morateck Patricia, Gavazova Stefana, Bellissimo Daniel B, Manco-Johnson Marilyn J, Gill Joan Cox, Montgomery Robert R
Abstract excerpt
Type 1 von Willebrand disease (VWD) is characterized by a partial quantitative deficiency of von Willebrand factor (VWF). Few VWF gene mutations have been identified that cause dominant type 1 VWD. The decreased survival of VWF in plasma has recently been identified as a novel mechanism for type 1 VWD. We report 4 families with moderately severe type 1 VWD characterized by low plasma VWF:Ag and FVIII:C levels,...
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