{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T13:46:52Z","timestamp":1776260812421,"version":"3.50.1"},"reference-count":14,"publisher":"Springer Science and Business Media LLC","issue":"11-12","license":[{"start":{"date-parts":[[2022,3,19]],"date-time":"2022-03-19T00:00:00Z","timestamp":1647648000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,3,19]],"date-time":"2022-03-19T00:00:00Z","timestamp":1647648000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100005788","name":"Medical University of Vienna","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100005788","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Wien Klin Wochenschr"],"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Acquired von Willebrand syndrome is exceedingly rare and accounts for only 1\u20133% of von Willebrand disease cases. In this short report, we present our own cases of acquired von Willebrand syndrome associated with monoclonal gammopathy. Both cases went into complete and sustained remission after intensive antimyeloma treatment. The first patient was not deemed fit for autologous stem cell transplantation and was managed with an extensive multidrug combination including daratumumab, carfilzomib, lenalidomide, cyclophosphamide and dexamethasone. After at least VGPR was achieved the coagulation studies rapidly normalized and remained normal after treatment de-escalation to lenalidomide\/dexamethasone maintenance. The second patient successfully underwent ASCT after 5\u00a0cycles of induction with daratumumab, bortezomib, cyclophosphamide and dexamethasone and has remained in full hematologic and hemostaseologic remission ever since.<\/jats:p><jats:p>The two cases highlight the efficacy of aggressive antimyeloma treatment in monoclonal gammopathy-associated acquired von Willebrand syndrome to achieve normalization of coagulation study, providing a\u00a0possible way to manage these patients.<\/jats:p>","DOI":"10.1007\/s00508-022-02012-3","type":"journal-article","created":{"date-parts":[[2022,3,19]],"date-time":"2022-03-19T09:02:21Z","timestamp":1647680541000},"page":"478-482","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Successful treatment of acquired von Willebrand syndrome associated with monoclonal gammopathy"],"prefix":"10.1007","volume":"134","author":[{"given":"Georg","family":"Jeryczynski","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hermine","family":"Agis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sabine","family":"Eichinger-Hasenauer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Theresa","family":"Krauth","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,3,19]]},"reference":[{"issue":"21","key":"2012_CR1","doi-asserted-by":"publisher","first-page":"2067","DOI":"10.1056\/NEJMra1601561","volume":"375","author":"FW Leebeek","year":"2016","unstructured":"Leebeek\u00a0FW, Eikenboom\u00a0JC. Von Willebrand\u2019s disease. N\u00a0Engl J Med. 2016;375(21):2067\u201380.","journal-title":"N Engl J Med"},{"issue":"2","key":"2012_CR2","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1053\/beha.2001.0141","volume":"14","author":"JJ Michiels","year":"2001","unstructured":"Michiels\u00a0JJ, Budde\u00a0U, van der Planken\u00a0M, et\u00a0al. Acquired von Willebrand syndromes: clinical features, aetiology, pathophysiology, classification and management. Best Pract Res Clin Haematol. 2001;14(2):401\u201336.","journal-title":"Best Pract Res Clin Haematol"},{"issue":"2","key":"2012_CR3","doi-asserted-by":"publisher","first-page":"356","DOI":"10.1002\/rth2.12481","volume":"5","author":"MY Abou-Ismail","year":"2021","unstructured":"Abou-Ismail\u00a0MY, Rodgers\u00a0GM, Bray\u00a0PF, et al. Acquired von Willebrand syndrome in monoclonal gammopathy\u2014a scoping review on hemostatic management. 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All studies performed were in accordance with the ethical standards indicated in each case. The patients provided informed consent to be included in this case report.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical standards"}}]}}