Acquired von Willebrand Disease in Multiple Myeloma - A Case Report and Review of Literature

Abraham SS, Abilash A and Sreejith GN

Department of Medical Oncology, Regional Cancer Centre, India

^*Correspondance to: Sreejith G Nair 

 PDF  Full Text Case Report | Open Access

Abstract:

Acquired von Willebrand Disease (avWD) is an exceedingly rare bleeding disorder in contrast to congenital von Willebrand Disease (vWD) with an estimated prevalence of 0.04% to 0.13% in the general population. It is commonly associated with underlying lymphoproliferative or myeloproliferative disorders like Monoclonal Gammopathy of Undetermined Significance (MGUS), Waldenström macroglobulinemia and multiple myeloma. We report a patient with multiple myeloma who developed severe avWD while undergoing autologous Peripheral Blood Stem Cell Transplant (PBST). A forty-nine-year-old gentleman with history of hypertension and no significant personal or family history of bleeding disorder was diagnosed with multiple myeloma (IgG kappa) ISS III. He attained Complete Response (CR) after 6 cycles of Bortezomib, Lenalidomide and Dexona (RVD) regimen and was scheduled for consolidation with High Dose Chemotherapy (HDCT) and autologous Peripheral Blood Stem Cell Transplant (PBST). Just before administration of stem cells, he developed bleeding from the subclavian catheter site. Evaluation of coagulopathy showed prolonged activated Partial Thromboplastin Time (aPTT), normal Prothrombin Time (PT), reduced vWF (16%) and factor VIII (20%) activity and normal factor IX and XI, suggestive of severe avWD. He was continued on maintenance chemotherapy and later underwent HDCT and PBST with blood product support (cryoprecipitate, fresh frozen plasma and apheresis platelets). He was also given intravenous immunoglobulin before catheter insertion. The procedure was uneventful. This subject had severe AvWD despite adequate treatment for multiple myeloma and showed good response to immunoglobulin. He underwent autologous PBST and never reported any further bleeding episode and is currently on follow up. Prompt identification and treatment of the underlying disorder is necessary apart from clinical management of avWD.

Keywords:

Acquired von Willebrand disease; Multiple myeloma; von Willebrand factor; Monoclonal gammopathy; Autologous peripheral blood stem cell transplant

Cite the Article:

Abraham SS, Abilash A, Sreejith GN. Acquired von Willebrand Disease in Multiple Myeloma - A Case Report and Review of Literature. Clin Oncol. 2023;8:2041..