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
