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Acquired von Willebrand syndrome (AvWS) is a rare condition most commonly associated with the lymphoproliferative disorders, including monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma, and Waldenström’s macroglobulinemia. It is a disorder of von Willebrand factor (vWF) concentration, structure, or function that is not inherited directly but is a consequence of another medical disorder. There are multiple mechanisms leading to AvWS: autoimmune clearance or inhibition of vWF, increased shear-induced proteolysis of vWF, or increased binding of vWF to platelets or other cell surfaces. Autoantibodies commonly are implicated in AvWS associated with MGUS. Thus, these patients often do not respond to standard von Willebrand disease (vWD) therapy. This case provided evidence that bortezomib may be an option for patients refractory to standard AvWS therapy in the setting of MGUS.

Bortezomib in the Treatment of Acquired von Willebrand Disease Secondary to Monoclonal Gammopathy of Undetermined Significance