Diagnosis and immediate treatment of acquired von Willebrand syndrome revealed by recurrent cerebral hemorrhage

Abstract

We report the case of a French woman with acquired von Willebrand syndrome who presents recurrent subarachnoid and intra-cerebral hemorrhage since 2012. She had no family or personal bleeding history. In the biologic explorations, APTT was abnormally high with no anticoagulant drugs (it was normal, historically). Two monoclonal IgG and IgM kappa proteins were detected without any lymphoproliferative disorder. Intravenous infusion of immunoglobulin is very effective in AVWS with immunoglobulin G monoclonal gammapathie of undetermined significance. We had a satisfactory correction of coagulation factors for about 30 days. The exploration of APTT is surely essential for the diagnosis and treatment.

Keywords: Acquired von Willebrand disease (AVWS); Hémorragies intra cérebrales spontanées; Immunoglobulines intraveineuses (IGIV); Intravenous immunoglobulins (IGIV); Maladie de Willebrand acquise (AVWS); Spontaneous intra-cerebral bleedings.