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Increased clearance of von Willebrand factor antigen post‐DDAVP in Type 1 von Willebrand disease: is it a potential pathogenic process?
Author(s) -
Brown S. A.,
Eldridge A.,
Collins P. W.,
Bowen D. J.
Publication year - 2003
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1046/j.1538-7836.2003.00359.x
Subject(s) - von willebrand factor , desmopressin , von willebrand disease , medicine , pathogenesis , endocrinology , antigen , vasopressin , coagulopathy , immunology , platelet
Summary. The mechanism of von Willebrand factor (VWF) clearance is not fully understood. The factors that affect VWF clearance, and the normal in vivo mechanism of clearance, may be relevant to the pathogenesis of Type 1 von Willebrand disease (VWD), in which there is a partial deficiency of VWF. In order to investigate the clearance of VWF in Type 1 VWD, the current study assessed the half‐life of VWF antigen ( t ½ VWF:Ag) in Type 1 VWD patients and individuals with mild hemophilia A following the administration of 1‐deamino‐8‐ d ‐arginine vasopressin (DDAVP; desmopressin). To date 20 individuals have been assessed, 13 with Type 1 VWD and seven with mild hemophilia A. The median t ½ VWF:Ag in the Type 1 VWD and mild hemophilia A groups were 4.6 h and 9.5 h, respectively. The difference between the t ½ VWF:Ag for the two groups was significant, P < 0.02. Analysis of the data showed a correlation between the t ½ VWF:Ag and the baseline VWF:Ag level prior to administration of DDAVP: lower baseline VWF:Ag levels were associated with a shorter t ½ VWF:Ag. These data suggest that increased clearance of VWF may be the pathogenic mechanism in some cases of Type 1 VWD.