{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,14]],"date-time":"2026-04-14T14:45:33Z","timestamp":1776177933036,"version":"3.50.1"},"reference-count":10,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2026,4,14]],"date-time":"2026-04-14T00:00:00Z","timestamp":1776124800000},"content-version":"vor","delay-in-days":13,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,4,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Von Willebrand disease (VWD) is the most common inherited coagulopathy, resulting from a deficiency of von Willebrand factor (VWF), which plays a central role in hemostasis. Type 3 VWD, the rarest and most severe form, is characterized by an almost complete absence of VWF, leading to severe and recurrent bleeding episodes. We report two pediatric cases of third-degree cousins, aged 3 and 2\u00a0years, born to consanguineous parents, who presented with severe hemorrhagic syndromes including profuse epistaxis, gingival bleeding, and anemia. Both had markedly prolonged activated partial thromboplastin time, reduced factor VIII, and severely diminished VWF activity and antigen levels, confirming the diagnosis of severe type 3 von Willebrand disease. Management included local hemostasis, avoidance of trauma, and prophylactic administration of plasma-derived VWF\/FVIII before invasive procedures. These cases highlight the diagnostic and therapeutic challenges of managing severe inherited bleeding disorders in resource-limited settings, emphasizing the importance of early recognition, family education, and structured follow-up.<\/jats:p>","DOI":"10.1093\/omcr\/omag037","type":"journal-article","created":{"date-parts":[[2026,2,25]],"date-time":"2026-02-25T12:45:49Z","timestamp":1772023549000},"source":"Crossref","is-referenced-by-count":0,"title":["Type 3 Von Willebrand disease: two clinical cases of a rare disorder"],"prefix":"10.1093","volume":"2026","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-0078-5141","authenticated-orcid":false,"given":"Khadija","family":"Belcadi Abassi","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Mohammed V Military Instruction Hospital , Avenue des FAR, Rabat 10100 ,","place":["Morocco"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karima","family":"Larbi Ouassou","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Mohammed V Military Instruction Hospital , Avenue des FAR, Rabat 10100 ,","place":["Morocco"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abdelilah","family":"Radi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Mohammed V Military Instruction Hospital , Avenue des FAR, Rabat 10100 ,","place":["Morocco"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Azzdine","family":"Laaraje","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Mohammed V Military Instruction Hospital , Avenue des FAR, Rabat 10100 ,","place":["Morocco"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara","family":"Babour","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Mohammed V Military Instruction Hospital , Avenue des FAR, Rabat 10100 ,","place":["Morocco"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Soukaina","family":"Ait Hmadouch","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Mohammed V Military Instruction Hospital , Avenue des FAR, Rabat 10100 ,","place":["Morocco"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amale","family":"Hassani","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Mohammed V Military Instruction Hospital , Avenue des FAR, Rabat 10100 ,","place":["Morocco"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammed E L","family":"Khorassani","sequence":"additional","affiliation":[{"name":"Hemophilia and Inherited Bleeding Disorders Treatment Center , Pediatric Hemato-Oncology Unit, Children\u2019s Hospital of Rabat, Rabat 10100 ,","place":["Morocco"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rachid","family":"Abilkassem","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Mohammed V Military Instruction Hospital , Avenue des FAR, Rabat 10100 ,","place":["Morocco"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2026,4,14]]},"reference":[{"key":"2026041409492895700_ref1","volume-title":"Maladie de Willebrand type 3 \u2013 Protocole national de diagnostic et de soins (PNDS)","author":"Haute Autorit\u00e9 de Sant\u00e9","year":"2022"},{"key":"2026041409492895700_ref2","doi-asserted-by":"publisher","first-page":"301","DOI":"10.1182\/bloodadvances.2020003265","article-title":"ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease","volume":"5","author":"James","year":"2021","journal-title":"Blood Adv"},{"key":"2026041409492895700_ref3","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1111\/hae.14970","article-title":"Diagnosis and treatment of von Willebrand disease in 2024 and beyond","volume":"30","author":"James","year":"2024","journal-title":"Haemophilia"},{"key":"2026041409492895700_ref4","doi-asserted-by":"publisher","first-page":"2067","DOI":"10.1056\/NEJMra1601561","article-title":"Von Willebrand\u2019s disease","volume":"375","author":"Leebeek","year":"2016","journal-title":"N Engl J Med"},{"key":"2026041409492895700_ref5","doi-asserted-by":"publisher","first-page":"2103","DOI":"10.1111\/j.1538-7836.2006.02146.x","article-title":"Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor","volume":"4","author":"Sadler","year":"2006","journal-title":"J Thromb Haemost"},{"key":"2026041409492895700_ref6","doi-asserted-by":"publisher","first-page":"171","DOI":"10.1111\/j.1365-2516.2007.01620.x","article-title":"Von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute expert panel report (USA)","volume":"14","author":"Nichols","year":"2008","journal-title":"Haemophilia"},{"key":"2026041409492895700_ref7","doi-asserted-by":"publisher","first-page":"683","DOI":"10.1056\/NEJMra040403","article-title":"Treatment of von Willebrand\u2019s disease","volume":"351","author":"Mannucci","year":"2004","journal-title":"N Engl J Med"},{"key":"2026041409492895700_ref8","doi-asserted-by":"publisher","first-page":"204","DOI":"10.1016\/j.jtha.2022.11.042","article-title":"Beyond the guidelines: how we approach challenging scenarios in the diagnosis and management of von Willebrand disease","volume":"21","author":"Abou-Ismail","year":"2023","journal-title":"J Thromb Haemost"},{"key":"2026041409492895700_ref9","doi-asserted-by":"publisher","first-page":"391","DOI":"10.2450\/2019.0183-18","article-title":"A phase III study comparing secondary long-term prophylaxis versus on-demand treatment with vWF\/FVIII concentrates in severe inherited von Willebrand disease","volume":"17","author":"Peyvandi","year":"2019","journal-title":"Blood Transfus"},{"key":"2026041409492895700_ref10","doi-asserted-by":"publisher","first-page":"2748","DOI":"10.1182\/bloodadvances.2019000656","article-title":"Efficacy of emicizumab in a pediatric patient with type 3 von Willebrand disease and alloantibodies","volume":"3","author":"Weyand","year":"2019","journal-title":"Blood Adv"}],"container-title":["Oxford Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/omcr\/article-pdf\/2026\/4\/omag037\/68078126\/omag037.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/omcr\/article-pdf\/2026\/4\/omag037\/68078126\/omag037.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,14]],"date-time":"2026-04-14T13:49:35Z","timestamp":1776174575000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/omcr\/article\/doi\/10.1093\/omcr\/omag037\/8653771"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4]]},"references-count":10,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026,4,12]]}},"URL":"https:\/\/doi.org\/10.1093\/omcr\/omag037","relation":{},"ISSN":["2053-8855"],"issn-type":[{"value":"2053-8855","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,4]]},"published":{"date-parts":[[2026,4]]},"article-number":"omag037"}}