{"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":1791567739922,"version":"4.3.4"},"reference-count":38,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,11,25]],"date-time":"2021-11-25T00:00:00Z","timestamp":1637798400000},"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":[[2022,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Since the approval of emicizumab,\u00a0a bispecific, factor VIII\u2010mimetic antibody, for use in persons with congenital haemophilia A in 2018, there have been increasing case reports and case series of off\u2010label use of emicizumab in other bleeding disorders, including acquired haemophilia A (AHA) and von Willebrand disease (VWD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>We conducted a scoping review on the use of emicizumab in AHA and VWD, focusing on the clinical presentation and outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a comprehensive search in PubMed, EMBASE and Scopus up to July 15, 2021. The following criteria were applied to the studies identified in the initial search: patients had a diagnosis of AHA or VWD; and the study reported on the clinical outcome of emicizumab use.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seventeen studies were included in the final review for a total of 41 patients (33 AHA, eight type 3 VWD). The majority of AHA patients and all type 3 VWD patients were started on emicizumab for active\/recurrent bleeds. The dosing regimen of emicizumab used varied significantly in AHA patients. All patients had a clinical response to emicizumab use. One AHA patient developed a stroke on emicizumab use in association with concomitant recombinant FVIIa use for surgery. Data on adverse events from emicizumab use were not specifically reported in 24.4% of patients (four AHA, six type 3 VWD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Based on published case reports and case series, emicizumab appears to be an effective haemostatic therapy for AHA and VWD. Larger confirmatory clinical trials are needed to confirm these findings.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14450","type":"journal-article","created":{"date-parts":[[2021,11,26]],"date-time":"2021-11-26T09:20:40Z","timestamp":1637918440000},"page":"4-17","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":46,"title":["Off\u2010label use of emicizumab in persons with acquired haemophilia A and von Willebrand disease: A scoping review of the literature"],"prefix":"10.1111","volume":"28","author":[{"given":"Vinay Mathew","family":"Thomas","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Division of Hematology and Hematologic Malignancies University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mouhamed Yazan","family":"Abou\u2010Ismail","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Division of Hematology and Hematologic Malignancies University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5208-3387","authenticated-orcid":false,"given":"Ming Y.","family":"Lim","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Division of Hematology and Hematologic Malignancies University of Utah  Salt Lake City Utah 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