Management of acquired von Willebrand syndrome

Le résumé fourni par la source

Acquired von Willebrand syndrome (AvWS) is a rare and often underdiagnosed bleeding disorder, estimated to affect up to 0.04% of the population. Unlike the hereditary form of von Willebrand disease (vWD), AvWS typically presents in adulthood in patients with no prior history of bleeding. First described in 1968 in a patient with systemic lupus erythematosus, AvWS is now increasingly recognized in association with hematologic malignancies (especially lymphoproliferative disorders), cardiovascular diseases (notably aortic stenosis and ventricular assist devices), and autoimmune conditions. The diagnosis remains challenging due to overlapping features with inherited vWD and the absence of a single definitive laboratory test. Key diagnostic steps include the assessment of von Willebrand factor antigen (vWF:Ag), factor VIII activity (FVIII:C), ristocetin cofactor activity (vWF:RCo), collagen binding assay (vWF:CB), and analysis of high-molecular-weight multimers (HMWM). The presence of an acquired inhibitor, shortened vWF half-life, or abnormal multimer pattern may support the diagnosis. Treatment should be directed at both the bleeding symptoms and the underlying disease. Therapeutic options include desmopressin, vWF/FVIII concentrates, intravenous immunoglobulin (IVIG), corticosteroids, and, in selected cases, plasmapheresis or immunosuppressive therapy. Definitive treatment of the comorbid condition (e.g., chemotherapy for lymphoma, valve replacement for aortic stenosis) often results in resolution of AvWS. In this article, we propose a practical diagnostic and therapeutic algorithm for clinicians managing patients with suspected or confirmed AvWS, with particular focus on patients with hematologic malignancies. Clinical scenarios, laboratory interpretation, and response-guided treatment strategies are also discussed.

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