Small intestinal angiodysplasia and hemorrhage associated with acquired von Willebrand syndrome type 2A secondary to multiple myeloma: Reversal after treatment with IRD regimen (a case report and a brief review of the literature)

Acquired von Willebrand syndrome (AVWS) is a rare acquired hemorrhagic disorder, characterized by qualitative or quantitative deficiencies in von Willebrand factor (VWF), which is easily misdiagnosed as a genetic von Willebrand disease (VWD) and probably underdiagnosed [1,2]. Usually, the diagnosis of AVWS is complicated, and both clinical and case history evaluation as well as laboratory results are required for discerning AVWS from inherited VWD [2,3]. Here we report a case of AVWS secondary to IgA-kappa type of multiple myeloma (MM), found that the existence of anti-VWF autoantibodies (IgG type) in plasma may contributed to the pathogenesis of AVWS which resulted into small intestinal angiodysplasia and hemorrhage, and successfully reversed the patient's bleeding tendency through multi-cycles of IRD regimen (ixazomib, lenalidomide, dexamethasone).
Source: Thrombosis Research - Category: Hematology Authors: Tags: Letter to the Editors-in-Chief Source Type: research