A role for intravenous immunoglobulin in the treatment of Acquired Von Willebrand Syndrome associated with IgM gammopathy.
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Acquired Von Willebrand Syndrome (AVWS) is a rare bleeding disorder resulting from reduction in plasma von Willebrand Factor (VWF) in patients with no previous personal or family history of Von Willebrand Disease (VWD). AVWS may present with significant bleeding, complicated by short-lived responses to DDAVP or VWF-containing concentrate. AVWS is commonly associated with cardiovascular and lymphoproliferative disorders (LPDs) . Amongst the LPDs, monoclonal gammopathy of undetermined significance (MGUS) is the most frequent association. Haemorrhage in MGUS-AVWS may be both spontaneous and severe. Treatment options include DDAVP, tranexamic acid and VWF-containing concentrates however patient co-morbidities, age or poor clinical and laboratory responses often restrict use . Furthermore, individual responses following DDAVP and VWF-containing concentrates are highly variable and response duration may be short . Consequently, the management of bleeding in patients with MGUS-AVWS presents a significant clinical challenge. Multiple second-line therapeutic approaches have been explored including intravenous immunoglobulin (IVIg) , plasmapheresis  and immunomodulatory drugs such as lenalidomide . While definitive treatment of the underlying LPD may induce AVWS remission, treatment for MGUS is not usually warranted.