{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T17:42:18Z","timestamp":1791567738541,"version":"4.3.4"},"reference-count":14,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,6,5]],"date-time":"2023-06-05T00:00:00Z","timestamp":1685923200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["pericles.pericles-gcp.literatumonline.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Emicizumab is approved to prevent bleeding in patients with congenital haemophilia A with or without inhibitors. However, no randomized trials addressed the efficacy of emicizumab in acquired haemophilia A (AHA).<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>To report the clinical and biochemical response of emicizumab in AHA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This single\u2010centre retrospective study included seven adults with AHA between November 2020 and May 2022. We collected patient characteristics, laboratory coagulation parameters, the use of haemostatic agents, bleeds and thrombotic events. Treatment was monitored using chromogenic FVIII assays. The assay with human reagents assesses both the emicizumab FVIII\u2010like\u2010activity and native patient FVIII\u2010activity. The assay with bovine reagents only measures the patients\u2019 native FVIII\u2010activity as emicizumab does not bind to bovine reagents.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients presented with spontaneous hematoma (<jats:italic>n<\/jats:italic>\u00a0=\u00a07), intramuscular bleeding (<jats:italic>n<\/jats:italic>\u00a0=\u00a02), haematuria (<jats:italic>n<\/jats:italic>\u00a0=\u00a02) and\/or gastro\u2010intestinal bleeding (<jats:italic>n<\/jats:italic>\u00a0=\u00a02). Six patients had major bleedings. At diagnosis, APTT was prolonged (91 seconds, IQR 73\u2013103), FVIII activity was 0% (IQR 0\u20131) and FVIII inhibitor 182 BU\/mL (IQR 104\u2013228). Emicizumab was administered weekly (3\u00a0mg\/kg) for 4 weeks, and thereafter every 2 weeks until regression of the inhibitor. Three patients received activated FVIIa (cumulative dose of 1.7\u00a0mg\/kg, IQR 1.2\u20102.2). All bleedings were controlled after treatment initiation, without further bleeds. After starting emicizumab, FVIII\u2010like activity reached \u22655% at 12 days (IQR 7\u201314), whereas recovery of the intrinsic FVIII\u2010activity \u22655% occurred at 128 days (IQR 88\u2013173), coinciding with the disappearance of the FVIII inhibitor. There were no safety issues.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In this AHA case series, no new clinically relevant bleeds were observed after initiation of emicizumab in conjunction with standard immunosuppressive therapy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14809","type":"journal-article","created":{"date-parts":[[2023,6,7]],"date-time":"2023-06-07T05:20:13Z","timestamp":1686115213000},"page":"1049-1055","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Emicizumab for acquired haemophilia A: A case series"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5364-7751","authenticated-orcid":false,"given":"Matthias M.","family":"Engelen","sequence":"first","affiliation":[{"name":"Hemophilia Center University Hospitals Leuven  Leuven Belgium"},{"name":"Center for Molecular and Vascular Biology Department of Cardiovascular Sciences University of Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johan","family":"Vandesande","sequence":"additional","affiliation":[{"name":"Hemophilia Center University Hospitals Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johan","family":"De Bent","sequence":"additional","affiliation":[{"name":"Hemophilia Center University Hospitals Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3105-6117","authenticated-orcid":false,"given":"Christine","family":"Van Laer","sequence":"additional","affiliation":[{"name":"Center for Molecular and Vascular Biology Department of Cardiovascular Sciences University of Leuven  Leuven Belgium"},{"name":"Department of Laboratory Medicine University Hospitals Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5052-9274","authenticated-orcid":false,"given":"Veerle","family":"Labarque","sequence":"additional","affiliation":[{"name":"Center for Molecular and Vascular Biology Department of Cardiovascular Sciences University of Leuven  Leuven Belgium"},{"name":"Department of Pediatric Hemato\u2010Oncology University Hospitals Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Jacquemin","sequence":"additional","affiliation":[{"name":"Center for Molecular and Vascular Biology Department of Cardiovascular Sciences University of Leuven  Leuven Belgium"},{"name":"Department of Laboratory Medicine University Hospitals Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathelijne","family":"Peerlinck","sequence":"additional","affiliation":[{"name":"Hemophilia Center University Hospitals Leuven  Leuven Belgium"},{"name":"Center for Molecular and Vascular Biology Department of Cardiovascular Sciences University of Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5429-8437","authenticated-orcid":false,"given":"Cedric","family":"Hermans","sequence":"additional","affiliation":[{"name":"Division of Hematology Hemostasis and Thrombosis Unit Cliniques universitaires Saint\u2010Luc Universit\u00e9 catholique de Louvain (UCLouvain)  Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8698-2858","authenticated-orcid":false,"given":"Peter","family":"Verhamme","sequence":"additional","affiliation":[{"name":"Hemophilia Center University Hospitals Leuven  Leuven Belgium"},{"name":"Center for Molecular and Vascular Biology Department of Cardiovascular Sciences University of Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7404-8918","authenticated-orcid":false,"given":"Thomas","family":"Vanassche","sequence":"additional","affiliation":[{"name":"Hemophilia Center University Hospitals Leuven  Leuven Belgium"},{"name":"Center for Molecular and Vascular Biology Department of Cardiovascular Sciences University of Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,6,5]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2019.230771"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2020002977"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12627"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1803550"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1703068"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0040-1717114"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14664"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14335"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020006315"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtha.2022.10.004"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1177\/20406207211038193"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2005.01204.x"},{"issue":"2","key":"e_1_2_10_14_1","first-page":"362","article-title":"A 4% solution of bovine serum albumin may be used in place of factor VIII:c deficient plasma in the control sample in the Nijmegen Modification of the Bethesda factor VIII:c inhibitor assay","volume":"88","author":"Verbruggen B","year":"2002","journal-title":"Thromb Haemost"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40259-018-0315-0"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.14809","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T12:46:59Z","timestamp":1693918019000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.14809"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,6,5]]},"references-count":14,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2023,7]]}},"alternative-id":["10.1111\/hae.14809"],"URL":"https:\/\/doi.org\/10.1111\/hae.14809","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,6,5]]},"assertion":[{"value":"2023-03-07","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-21","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-06-05","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}