DDAVP in type IIa von Willebrand's disease
HR Gralnick, SB Williams, LP McKeown, ME Rick, P Maisonneuve, C Jenneau and Y Sultan
1-D-Amino(8-D-arginine)-vasopressin (DDAVP) infusion in three patients with
type IIa von Willebrand's disease (vWD) resulted in a normalization of the
factor VIII coagulant, factor VIII-related antigen, and von Willebrand
factor (vWF) (ristocetin cofactor) activities and the bleeding time. The
normalization of these hemostatic parameters persisted for four hours. Over
the same time period there was a marked increase in the quantity of the vWF
multimers when blood was collected in the presence of protease inhibitors.
The vWF multimers present were even larger than the normal. When blood was
collected in the absence of protease inhibitors, a smaller increase in the
plasma vWF multimers was observed and fewer of the intermediate and larger
vWF multimers were seen; multimers larger than those present in normal
plasma were not visualized. The platelet vWF multimers and activities did
not change with or without inhibitors. These studies suggest that there is
a subgroup of patients with type IIa vWD who respond to DDAVP with complete
normalization of their hemostatic abnormalities and whose vWF is sensitive
to proteolysis.
Volume 67,
Issue 2,
pp. 465-468,
02/01/1986
Copyright © 1986 by The American Society of Hematology
