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Prepublished online as a Blood First Edition Paper on September 19, 2002; DOI 10.1182/blood-2002-03-0814.
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Blood, 1 February 2003, Vol. 101, No. 3, pp. 946-948
HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY
Brief report
Changes in von Willebrand factor-cleaving protease
(ADAMTS13) activity after infusion of desmopressin
Rosemarie A. Reiter,
Paul Knöbl,
Katalin Varadi, and
Peter L. Turecek
From the Department of Clinical Pharmacology and the
Department of Medicine 1, University of Vienna; Baxter Bio Science,
Vienna, Austria.
von Willebrand factor-cleaving protease (ADAMTS13) cleaves von
Willebrand factor (VWF) and regulates its physiologic function. To
investigate the relation between ADAMTS13 activity and VWF, we compared
ADAMTS13 activity with the VWF-related parameters VWF antigen (VWF:Ag),
VWF collagen-binding activity (VWF:CBA), VWF-propeptide, proVWF, and
VWF multimeric composition in 10 healthy volunteers and 3 patients with
type 1 von Willebrand disease before and after infusing 0.3 µg/kg
desmopressin. The VWF-related parameters in the volunteers increased 60 minutes after start of infusion by 3.7-fold for VWF:Ag, 7.2-fold for
propeptide, and 2.2-fold for VWF:CBA. Unusually large VWF multimers and
traces of proVWF appeared. The ADAMTS13 activity decreased to about
half the initial value. After 24 hours values returned to baseline.
Patients with type 1 von Willebrand disease showed similar results. We
conclude that the inverse correlation between ADAMTS13 and VWF-related parameters suggests a consumption of ADAMTS13 after the
desmopressin-induced release of higher multimers of VWF.
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