Acquired von Willebrand disease associated with free lambda light chain monoclonal gammopathy, normal bleeding time and response to prednisone

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We report a case of acquired von Willebrand's syndrome with severe gastrointestinal bleeding and associated free monoclonal lambda light chains. The patient had a rapid sustained clinical and laboratory response to the administration of prednisone. Of note in this patient was the occurrence of angiodysplasia which has previously been reported in association with acquired von Willebrand's syndrome. No inhibitors of VWF:Ag, VWF:RCoF, or factor VIII:C were detected by mixing studies and the bleeding time was normal. Very few high molecular weight von Willebrand multimers were present prior to prednisone; however, the pattern reverted to a normal distribution following treatment. In appropriate patients with acquired von Willebrand's syndrome and monoclonal para-proteins, a trial of prednisone may be indicated.