{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T00:49:48Z","timestamp":1793321388720,"version":"4.5.0"},"reference-count":54,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2018,11,15]],"date-time":"2018-11-15T00:00:00Z","timestamp":1542240000000},"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":[[2019,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Emicizumab\u2010kywh (ACE910) is a recombinant, humanized, asymmetric bispecific antibody that functions to bring activated FIX (FIXa) and zymogen FX into an appropriate steric conformation to medicate the activation of FX to FXa thereby mimicking the cofactor function of FVIIIa.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>The objective of this manuscript was to review the development and potential role for emicizumab in the treatment of patients with haemophilia A with and without inhibitors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A Cochrane Library and PubMed (MEDLINE) search focusing on emicizumab in haemophilia was conducted.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 37 citations were retrieved and serve as the database for the literature reviewed herein. Once\u2010weekly subcutaneous injection of emicizumab at three dose levels has been shown to be effective as prophylaxis to prevent bleeding in a majority haemophilia A patients with inhibitors to FVIII. Likewise, prevention of bleeding was also observed in more than two thirds of patients without inhibitors to FVIII. One antidrug antibody to emicizumab has been reported in over 600 treated patients, two have developed thromboembolic events and three thrombotic microangiopathy. These thrombotic complications have occurred in conjunction with FVIII\u2010bypassing agents, and none have been observed following recommendations from the manufacturer regarding concomitant use of bypassing agents. The median annual treated bleeding rates were decreased in patients with as well as those without an inhibitor to FVIII.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The principal advantage of emicizumab is subcutaneous administration and effectiveness irrespective of the presence of inhibitors. Emicizumab could conceivably represent a new epoch in the treatment of people with haemophilia A.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.13641","type":"journal-article","created":{"date-parts":[[2018,11,15]],"date-time":"2018-11-15T21:54:41Z","timestamp":1542318881000},"page":"11-20","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":48,"title":["Emicizumab: Review of the literature and critical appraisal"],"prefix":"10.1111","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6360-0113","authenticated-orcid":false,"given":"E. Carlos","family":"Rodriguez\u2010Merchan","sequence":"first","affiliation":[{"name":"Department of Orthopaedic Surgery La Paz University Hospital\u2010IdiPaz Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0420-8565","authenticated-orcid":false,"given":"Leonard A.","family":"Valentino","sequence":"additional","affiliation":[{"name":"Rush University Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,11,15]]},"reference":[{"key":"e_1_2_6_2_1","volume-title":"Report on the Annual Global Survey 2016","author":"WFH","year":"2017"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200106073442307"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1542\/pir.34-7-289"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.01776.x"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13361"},{"key":"e_1_2_6_7_1","unstructured":"OldenburgJ KhairK MazzucconiMG et al.Real World prospective data on bleeding frequency in 1 000 patients with hemophilia A \u2013 is the goal of zero bleeds achievable?Paper presented at: 10th Annual Congress of the European Association for Haemophilia and Allied Disorders 2017(1\u20103) pp. February 2017. 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