{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T11:25:05Z","timestamp":1785410705708,"version":"3.56.0"},"reference-count":27,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2018,10,8]],"date-time":"2018-10-08T00:00:00Z","timestamp":1538956800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007013","name":"F. Hoffmann-La Roche","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007013","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Prospectively collected, real\u2010world data on bleeds, haemophilic treatment and safety outcomes in persons with haemophilia A (PwHA) with factor VIII (FVIII) inhibitors are limited. A prospective, global, multi\u2010centre, non\u2010interventional study (NIS; NCT02476942) collected detailed real\u2010world data in PwHA treated per local routine clinical practice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To characterize bleeding rates, haemophilic treatment practices, prophylaxis adherence and adverse events (AEs) in adult\/adolescent PwHA with inhibitors in the NIS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Participants aged \u226512\u00a0years with congenital haemophilia A\/documented high\u2010titre FVIII inhibitor history were enrolled. Participants remained on their usual treatment; no interventions were applied.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Overall, 103 PwHA with inhibitors enrolled, (median [range] age 31 [12\u201075] years) and were monitored for median (range) 26.0 (4.1\u201069.6) weeks. In the episodic (n\u00a0=\u00a075) and prophylactic (n\u00a0=\u00a028) treatment groups, respectively, 1244 and 325 bleeds were reported, and 528 (42.4%) and 104 (32.0%) were not treated; annualized bleeding rates (ABRs; 95% confidence interval) were 18.6 (15.2\u201022.8) and 14.9 (10.5\u201021.2) for treated bleeds, and 32.7 (27.3\u201039.1) and 25.0 (18.4\u201034.0) for all bleeds. Coagulation products used included activated prothrombin complex concentrate (aPCC) and\/or recombinant activated FVII. Among participants prescribed aPCC prophylaxis, 35.0% adhered to both prescribed frequency of aPCC administration and prescribed dose. Serious AEs of haemarthrosis and muscle haemorrhage were reported; most common AEs were arthralgia, viral upper respiratory tract infection and pyrexia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>ABRs (treated bleeds and all bleeds) remain high on standard treatment; this prospective NIS demonstrates the need for more effective treatments for PwHA with inhibitors to reduce\/prevent bleeds, with potential to improve prophylaxis adherence and further improve outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.13612","type":"journal-article","created":{"date-parts":[[2018,10,8]],"date-time":"2018-10-08T10:00:53Z","timestamp":1538992853000},"page":"921-929","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Bleeding events and safety outcomes in persons with haemophilia A with inhibitors: A prospective, multi\u2010centre, non\u2010interventional study"],"prefix":"10.1111","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5781-7669","authenticated-orcid":false,"given":"Johnny","family":"Mahlangu","sequence":"first","affiliation":[{"name":"Haemophilia Comprehensive Care Centre, Faculty of Health Sciences University of the Witwatersrand and NHLS Johannesburg South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3038-2145","authenticated-orcid":false,"given":"Johannes","family":"Oldenburg","sequence":"additional","affiliation":[{"name":"Department of Experimental Haematology and Transfusion Medicine University Clinic Bonn Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8742-0275","authenticated-orcid":false,"given":"Michael U.","family":"Callaghan","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital of Michigan Detroit Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Midori","family":"Shima","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nara Medical University Kashihara, Nara Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Santagostino","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda Foundation, Ospedale Maggiore Policlinico Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maggie","family":"Moore","sequence":"additional","affiliation":[{"name":"Washington Center for Bleeding Disorders at Bloodworks Northwest Seattle Washington"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Recht","sequence":"additional","affiliation":[{"name":"Oregon Health &amp; Science University Portland Oregon"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Garcia","sequence":"additional","affiliation":[{"name":"Haematology Department Hospital Mexico San Jose Costa Rica"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renchi","family":"Yang","sequence":"additional","affiliation":[{"name":"Institute of Hematology and Blood Diseases Hospital Chinese Academy of Medical Sciences Tianjin China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Lehle","sequence":"additional","affiliation":[{"name":"F. 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South San Francisco California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6547-5240","authenticated-orcid":false,"given":"Rebecca","family":"Kruse\u2010Jarres","sequence":"additional","affiliation":[{"name":"Washington Center for Bleeding Disorders at Bloodworks Northwest Seattle Washington"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,10,8]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200106073442307"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.5581\/1516-8484.20130108"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11606-014-2893-y"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.2147\/TCRM.S4412"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2003.00780.x"},{"key":"e_1_2_8_7_1","unstructured":"World Federation of Hemophilia.About bleeding disorders. 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