{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T17:42:19Z","timestamp":1791567739968,"version":"4.3.4"},"reference-count":19,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,9,26]],"date-time":"2022-09-26T00:00:00Z","timestamp":1664150400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2023,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Acquired haemophilia A (AHA) is a rare and potentially life\u2010threatening bleeding disorder arising from autoantibodies that inhibit coagulation factor VIII (FVIII). Treatment entails achieving haemostasis with bypassing agents or factor replacement, and eradication of the inhibitor with immunosuppressive therapy (IST). Due to the rarity of AHA, there are few prospective data to guide management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We present a retrospective report of 11 AHA patients treated with emicizumab, a FVIII\u2010mimetic bispecific antibody, administered at 3\u00a0mg\/kg weekly for 4 weeks in conjunction with rituximab\u2010based immunosuppressive therapy. The chromogenic FVIII inhibitor assay was used to assess for inhibitor eradication.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The median follow\u2010up was 13.9 months. The median number of days of additional haemostatic therapy or red blood cell transfusions after initiating emicizumab was 2 (range 0\u201315). The median was 0 days (range 0\u20138) for patients who did not require vascular embolization to achieve haemostasis. Eight patients achieved a complete remission (defined as recovery of FVIII activity to\u00a0&gt;\u00a050% with a negative inhibitor test in the absence of haemostatic and IST); two patients achieved a partial remission (FVIII activity\u00a0&gt;\u00a050% but with detectable inhibitor); one patient experienced refractory disease. One patient experienced rebleeding and two patients experienced inhibitor recurrence. No thrombotic, thrombotic microangiopathic or infectious complications occurred.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our observations suggest emicizumab can facilitate haemostasis for AHA patients and be combined with safer, lower\u2010intensity immunosuppressive therapies to achieve remission.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14664","type":"journal-article","created":{"date-parts":[[2022,9,27]],"date-time":"2022-09-27T04:24:15Z","timestamp":1664252655000},"page":"84-89","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Emicizumab for the treatment of acquired hemophilia A: Retrospective review of a single\u2010institution experience"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9218-215X","authenticated-orcid":false,"given":"Evan C.","family":"Chen","sequence":"first","affiliation":[{"name":"Dana Farber Cancer Institute  Boston Massachusetts USA"},{"name":"Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William","family":"Gibson","sequence":"additional","affiliation":[{"name":"Dana Farber Cancer Institute  Boston Massachusetts USA"},{"name":"Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula","family":"Temoczko","sequence":"additional","affiliation":[{"name":"Division of Hematology Brigham and Women's Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4100-7826","authenticated-orcid":false,"given":"Nathan T.","family":"Connell","sequence":"additional","affiliation":[{"name":"Division of Hematology Brigham and Women's Hospital  Boston Massachusetts USA"},{"name":"Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Handin","sequence":"additional","affiliation":[{"name":"Division of Hematology Brigham and Women's Hospital  Boston Massachusetts USA"},{"name":"Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1981-1677","authenticated-orcid":false,"given":"Aric D.","family":"Parnes","sequence":"additional","affiliation":[{"name":"Division of Hematology Brigham and Women's Hospital  Boston Massachusetts USA"},{"name":"Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,9,26]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-06-029850"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2003.04731.x"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04654.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12138"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0000000000000582"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1650169"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-02-408930"},{"issue":"4","key":"e_1_2_8_9_1","first-page":"Ra55","article-title":"Drug\u2010induced anti\u2010factor VIII antibodies: a systematic review","volume":"13","author":"Franchini M","year":"2007","journal-title":"Med Sci 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