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Updated: Jan 19, 2026

Investigating von Willebrand Factor Pathophysiology Using a Flow Chamber Model of von Willebrand Factor-platelet String Formation
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A Prolonged Treatment Response in Acquired Von Willebrand Syndrome

Stéphanie Désage1, Sandra Le Quellec2, Lionel Karlin3

  • 1Laboratoire d'hématologie, Centre de Biologie et de Pathologie Est, Hospices Civils de Lyon, Lyon, France.

Hamostaseologie
|September 24, 2019
PubMed

Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder. We report herein a case of AVWS due to a monoclonal gammopathy of undetermined significance, in which a transient but prolonged response to a treatment by intravenous immunoglobulin (IVIG) was observed. The diagnosis was fortuitously made in a preoperative setting for neurosurgery, after biological exploration of an isolated prolonged activated partial thromboplastin time. AVWS was confirmed by an accelerated clearance of an infused plasma-derived von Willebrand factor (VWF) concentrate. High doses of IVIG were used to perform the neurosurgery. Fifty-four days after IVIG, the patient was still responding to treatment with normal levels of factor VIII and VWF.

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