A Prolonged Treatment Response in Acquired Von Willebrand Syndrome

Abstract

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.

Keywords

acquired von Willebrand syndrome - intravenous immunoglobulins - monoclonal gammopathy of undetermined significance - management

Author Contributions

All authors had access to the data and play a role in writing the manuscript. Lucia Rugeri and Lionel Karlin participated in the clinical management of the patient. Stéphanie Désage wrote the manuscript. Sandra Le Quellec, Anne Lienhart, Sandrine Meunier, and Lucia Rugeri critically read and edited the manuscript.

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