| Literature DB >> 33504531 |
Chintan P Shah1, Jess Delaune2, Molly W Mandernach2.
Abstract
Acquired von Willebrand syndrome is a rare bleeding disorder characterised by a later age of onset without a personal or family history of bleeding diathesis. It is vital to discern acquired von Willebrand syndrome from inherited von Willebrand disease and other acquired bleeding disorders as management differs significantly. Acquired von Willebrand syndrome is usually secondary to an underlying disorder such as lymphoproliferative disorder, myeloproliferative neoplasm, solid tumour, cardiovascular disorder, autoimmune disorders or hypothyroidism. Diagnosis is often delayed with a significant risk of morbidity and even mortality. Here we present a case of a 74-year-old man with an acquired bleeding disorder and work up suggestive of acquired von Willebrand syndrome secondary to immunoglobulin G kappa multiple myeloma. He was treated successfully with intravenous immunoglobulin, von Willebrand Factor/Coagulation Factor VIII Complex (human), myeloma directed chemotherapy and autologous stem cell transplantation. We also discuss the management strategies that are largely based on retrospective studies and case reports. © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.Entities:
Keywords: haematology (drugs and medicines); malignant and benign haematology
Year: 2021 PMID: 33504531 PMCID: PMC7843347 DOI: 10.1136/bcr-2020-239053
Source DB: PubMed Journal: BMJ Case Rep ISSN: 1757-790X