Successful treatment of acquired von Willebrand syndrome in multiple myeloma

Abstract

Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder characterized by quantitative or qualitative defects of the von Willebrand factor (vWF) with laboratory findings and clinical presentations similar to those of inherited von Willebrand disease (vWD). In contrast to the inherited form, the bleeding disorder is not due to genetic defects of von Willebrand factor. The prevalence of AVWS is poorly defined. Data from reviews, international registry and reference laboratories in Germany suggest a rate of approximately one case of AVWS in every 30–40 samples from patients with confirmed bleeding disorders. An association with different disorders has been described.

Multiple myeloma (MM) is a malignant plasma cell disorder defined by the accumulation of monoclonal plasma cells in the bone marrow or extramedullary lesions and often accompanied with a monoclonal paraprotein in blood and/or urine.

Symptomatic myeloma disease requiring treatment is usually characterized by renal failure, anemia or destructive bone lesions. Bleeding due to AVWS as a leading symptom of the disease is uncommon.

Here we report the case of a patient with MM presenting with significant bleeding from AVWS as primary symptom. Bleeding lasted for several years before diagnostic work-up including a bone marrow puncture revealed MM. Myeloma-specific treatment resulted in an adequate hematological response characterized by decreasing monoclonal paraprotein-levels and AVWS was resolved.