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Prepublished online as a Blood First Edition Paper on September 19, 2002; DOI 10.1182/blood-2002-03-0814.
Submitted March 15, 2002
Accepted September 6, 2002
Changes in von Willebrand factor-cleaving protease (ADAMTS13) activity after infusion of desmopressin
Rosemarie A Reiter, Paul Knoebl*, Katalin Varadi, and Peter L Turecek
Department of Clinical Pharmacology, University of Vienna, Vienna, Austria
Department of Medicine 1, University of Vienna, Vienna, Austria
Baxter Bio Science, Vienna, Austria
* Corresponding author; email: paul.knoebl{at}akh-wien.ac.at.
Von Willebrand factor-cleaving protease (ADAMTS13) cleaves von Willebrand factor (VWF) and regulates its physiological 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 ten healthy volunteers and three 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, 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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