DDAVP in type IIa von Willebrand's disease.

Abstract:

: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.

journal_name

Blood

journal_title

Blood

authors

Gralnick HR,Williams SB,McKeown LP,Rick ME,Maisonneuve P,Jenneau C,Sultan Y

subject

Has Abstract

pub_date

1986-02-01 00:00:00

pages

465-8

issue

2

eissn

0006-4971

issn

1528-0020

journal_volume

67

pub_type

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