{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T22:58:10Z","timestamp":1776812290319,"version":"3.51.2"},"reference-count":9,"publisher":"Georg Thieme Verlag KG","issue":"02","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hamostaseologie"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Therapeutic management of acquired von Willebrand syndrome (AVWS) can be challenging, particularly in cases of AVWS associated with monoclonal IgM such as Waldenstr\u00f6m macroglobulinemia (WM) where several therapeutic options may be ineffective. Here, we describe the case of an 88-year-old patient who developed AVWS during follow-up for WM. The presence of a severe bleeding symptomatology not controlled by several therapies (plasma-derived von Willebrand factor, plasmapheresis) led us to introduce a supplementation with recombinant von Willebrand factor, vonicog \u03b1 (Veyvondi, Takeda, Japan), starting at a dose of 50\u2009IU\/kg\/d. This supplementation allowed clinical (no further bleeding) and biological (hemoglobin level, von Willebrand factor parameters) improvements. Because of the persistence of bleeding risk factors, the treatment was maintained at a prophylactic dose (20 UI\/kg three times a week), without recurrence of bleeding events for a period of 9 months.<\/jats:p>","DOI":"10.1055\/a-2266-7984","type":"journal-article","created":{"date-parts":[[2024,3,22]],"date-time":"2024-03-22T20:31:35Z","timestamp":1711139495000},"page":"111-114","source":"Crossref","is-referenced-by-count":1,"title":["Use of Vonicog Alpha and Acquired von Willebrand Syndrome, a New Approach: A Case Report"],"prefix":"10.1055","volume":"46","author":[{"given":"Adeline","family":"Blandini\u00e8res","sequence":"additional","affiliation":[{"name":"Department of Hematology, Public Assistance Hospitals of Paris (AP-HP), Bic\u00eatre Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"University of Paris-Saclay, INSERM Hemostasis, Inflammation and Thrombosis Unit (HITh) U1176, Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sophie","family":"Combe","sequence":"additional","affiliation":[{"name":"Department of Hematology, Public Assistance Hospitals of Paris (AP-HP), Bic\u00eatre Hospital, Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"No\u00e9mie","family":"Chanson","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Clinical Immunology, Public Assistance Hospitals of Paris (AP-HP), Bic\u00eatre Hospital, Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olivier","family":"Lambotte","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Clinical Immunology, Public Assistance Hospitals of Paris (AP-HP), Bic\u00eatre Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"University of Paris-Saclay, INSERM, CEA, UMR 1184, Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9cile","family":"Lavenu-Bombled","sequence":"additional","affiliation":[{"name":"Department of Hematology, Public Assistance Hospitals of Paris (AP-HP), Bic\u00eatre Hospital, Le Kremlin-Bic\u00eatre, France"},{"name":"University of Paris-Saclay, INSERM Hemostasis, Inflammation and Thrombosis Unit (HITh) U1176, Le Kremlin-Bic\u00eatre, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2024,3,22]]},"reference":[{"issue":"08","key":"ref1","doi-asserted-by":"crossref","first-page":"2032","DOI":"10.3324\/haematol.2020.255117","article-title":"Acquired von Willebrand syndrome: focused for hematologists","volume":"105","author":"M Franchini","year":"2020","journal-title":"Haematologica"},{"issue":"02","key":"ref2","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1517\/13543784.9.2.347","article-title":"Therapeutic approaches to acquired von 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Recombinant von Willebrand Factor in One Patient","volume":"145","author":"M H\u00f6pting","year":"2022","journal-title":"Acta Haematol"},{"issue":"01","key":"ref6","doi-asserted-by":"crossref","first-page":"e76","DOI":"10.1055\/a-2008-4367","article-title":"Treatment of acquired von Willebrand disease due to extracorporeal membrane oxygenation in a pediatric COVID-19 patient with vonicog alfa: a case report and literature review","volume":"7","author":"L Heubner","year":"2023","journal-title":"TH Open"},{"issue":"01","key":"ref7","doi-asserted-by":"crossref","first-page":"e22","DOI":"10.1111\/hae.13374","article-title":"A role for intravenous immunoglobulin in the treatment of acquired von Willebrand syndrome associated with IgM gammopathy","volume":"24","author":"M Lavin","year":"2018","journal-title":"Haemophilia"},{"issue":"14","key":"ref8","doi-asserted-by":"crossref","first-page":"2813","DOI":"10.1182\/bloodadvances.2021004843","article-title":"Continuous-infusion von Willebrand factor concentrate is effective for the management of acquired von Willebrand disease","volume":"5","author":"K E Dane","year":"2021","journal-title":"Blood Adv"},{"issue":"21","key":"ref9","doi-asserted-by":"crossref","first-page":"3481","DOI":"10.1182\/bloodadvances.2019000368","article-title":"New therapies for von Willebrand disease","volume":"3","author":"P M Mannucci","year":"2019","journal-title":"Blood Adv"}],"container-title":["H\u00e4mostaseologie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2266-7984.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,21]],"date-time":"2026-04-21T22:30:47Z","timestamp":1776810647000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2266-7984"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,22]]},"references-count":9,"journal-issue":{"issue":"02","published-online":{"date-parts":[[2026,4,21]]},"published-print":{"date-parts":[[2026,4]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2266-7984","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0720-9355","2567-5761"],"issn-type":[{"value":"0720-9355","type":"print"},{"value":"2567-5761","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,3,22]]}}}