{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T07:18:23Z","timestamp":1763018303620,"version":"3.44.0"},"reference-count":19,"publisher":"BMJ","issue":"1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2021,1,7]]},"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.1136\/bcr-2020-239053","type":"journal-article","created":{"date-parts":[[2021,1,27]],"date-time":"2021-01-27T12:38:15Z","timestamp":1611751095000},"page":"e239053","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Acquired von Willebrand syndrome in association with multiple myeloma: remission after stem cell transplant"],"prefix":"10.1136","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2441-908X","authenticated-orcid":false,"given":"Chintan P","family":"Shah","sequence":"first","affiliation":[{"name":"Divison of Hematology\/Oncology, Department of Medicine, University of Florida, Gainesville, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jess","family":"Delaune","sequence":"additional","affiliation":[{"name":"Divison of Hematology\/Oncology, Department of Medicine, University of Florida, Gainesville, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Molly W","family":"Mandernach","sequence":"additional","affiliation":[{"name":"Divison of Hematology\/Oncology, Department of Medicine, University of Florida, Gainesville, Florida, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2021,1,27]]},"reference":[{"key":"2025082711081424000_14.1.e239053.1","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.7.2.8440408"},{"key":"2025082711081424000_14.1.e239053.2","doi-asserted-by":"crossref","first-page":"1337","DOI":"10.17219\/acem\/64942","article-title":"Acquired von Willebrand syndrome","volume":"25","author":"Mital","year":"2016","journal-title":"Adv Clin Exp Med"},{"key":"2025082711081424000_14.1.e239053.3","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1055\/s-0037-1614018","article-title":"Acquired von Willebrand syndrome: data from an international registry","volume":"84","author":"Federici","year":"2000","journal-title":"Thromb Haemost"},{"key":"2025082711081424000_14.1.e239053.4","doi-asserted-by":"crossref","first-page":"1295","DOI":"10.1515\/jpem-2019-0082","article-title":"Acquired von Willebrand's syndrome caused by primary hypothyroidism in a 5-year-old girl","volume":"32","author":"Flot","year":"2019","journal-title":"J Pediatr Endocrinol Metab"},{"key":"2025082711081424000_14.1.e239053.5","doi-asserted-by":"crossref","unstructured":"Fricke WA , Brinkhous KM , Garris JB . Comparison of inhibitory and binding characteristics of an antibody causing acquired von Willebrand syndrome: an assay for von Willebrand factor binding by antibody 1985:562\u20139.","DOI":"10.1182\/blood.V66.3.562.bloodjournal663562"},{"key":"2025082711081424000_14.1.e239053.6","doi-asserted-by":"crossref","unstructured":"Mannucci PM , Lombardi R , Bader R . Studies of the pathophysiology of acquired von Willebrand\u2019s disease in seven patients with lymphoproliferative disorders or benign monoclonal gammopathies 1984:614\u201321.","DOI":"10.1182\/blood.V64.3.614.bloodjournal643614"},{"key":"2025082711081424000_14.1.e239053.7","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/87.5.663"},{"key":"2025082711081424000_14.1.e239053.8","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830350210"},{"key":"2025082711081424000_14.1.e239053.9","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2010.05.016"},{"key":"2025082711081424000_14.1.e239053.10","doi-asserted-by":"publisher","DOI":"10.1177\/107602960100700206"},{"key":"2025082711081424000_14.1.e239053.11","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(199702)54:2<139::AID-AJH7>3.0.CO;2-Y"},{"key":"2025082711081424000_14.1.e239053.12","doi-asserted-by":"crossref","first-page":"2707","DOI":"10.1182\/blood.V92.8.2707","article-title":"Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches","volume":"92","author":"Federici","year":"1998","journal-title":"Blood"},{"key":"2025082711081424000_14.1.e239053.13","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1055\/s-0037-1615752","article-title":"Pharmacokinetic studies with FVIII\/von Willebrand factor concentrate can be a diagnostic tool to distinguish between subgroups of patients with acquired von Willebrand syndrome","volume":"85","author":"Luboshitz","year":"2001","journal-title":"Thromb Haemost"},{"key":"2025082711081424000_14.1.e239053.14","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1007\/s00277-016-2650-x","article-title":"Distinct mechanisms account for acquired von Willebrand syndrome in plasma cell dyscrasias","volume":"95","author":"Dicke","year":"2016","journal-title":"Ann Hematol"},{"key":"2025082711081424000_14.1.e239053.15","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1097\/MD.0b013e3182397166","article-title":"Acquired von Willebrand syndrome associated with monoclonal gammopathy: a single-center study of 36 patients","volume":"90","author":"Voisin","year":"2011","journal-title":"Medicine"},{"key":"2025082711081424000_14.1.e239053.16","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-8411(05)80020-4"},{"key":"2025082711081424000_14.1.e239053.17","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1111\/j.1365-2141.1992.tb04610.x","article-title":"Efficacy of high-dose intravenous gammaglobulin in the management of acquired von Willebrand's disease during orthopaedic surgery","volume":"82","author":"Gross","year":"1992","journal-title":"Br J Haematol"},{"key":"2025082711081424000_14.1.e239053.18","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1002\/ajh.2830410212","article-title":"Effectiveness of high-dose intravenous immunoglobulin in a case of acquired von Willebrand syndrome with chronic melena not responsive to desmopressin and factor VIII concentrate","volume":"41","author":"Castaman","year":"1992","journal-title":"Am J Hematol"},{"key":"2025082711081424000_14.1.e239053.19","first-page":"1","article-title":"Treatment of acquired von Willebrand syndrome and prevention of bleeding postautologous stem cell transplant during severe pancytopenia with IVIg","volume":"2015","author":"Zoghi","year":"2015","journal-title":"Case Rep Hematol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2020-239053","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T18:08:23Z","timestamp":1756318103000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2020-239053"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1]]},"references-count":19,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,1,27]]},"published-print":{"date-parts":[[2021,1]]}},"alternative-id":["10.1136\/bcr-2020-239053"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2020-239053","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2021,1]]}}}