{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,20]],"date-time":"2026-03-20T00:55:30Z","timestamp":1773968130013,"version":"3.50.1"},"reference-count":33,"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>The development of inhibitors against factor FIX (FIX) is the most serious complication of FIX replacement therapy in haemophilia B (HB) patients. Currently, only few cohorts of HB inhibitor patients have been reported worldwide.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>This Chinese nationwide study of HB inhibitor patients explored their risk factors for FIX inhibitor development and experience on their management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively analysed patient characteristics, F9 genotypes, treatment strategies and outcomes of HB inhibitor patients registered to the Chinese National Registry and Patient Organization Registry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010four unique HB inhibitor patients were identified in 4485 unique HB patients registered by year 2021 to the two Registries. Inhibitor diagnosis were usually delayed and the low prevalence (.98%) may suggest some inhibitor patients were not identified. Their median age at inhibitor diagnosis was 7.5 (IQR, 3.0\u201314.8) years. Most patients (95.5%) had high\u2010titre inhibitors. Allergic\/Anaphylactic reactions occurred in 59.1% patients. Large deletions and nonsense mutations were the most common <jats:italic>F9<\/jats:italic> mutation types in our FIX inhibitor patients. Patients with large <jats:italic>F9<\/jats:italic> gene deletions were more likely to develop inhibitors (<jats:italic>p<\/jats:italic> =\u00a0.0002), while those with missense mutations had a low risk (<jats:italic>p<\/jats:italic> &lt;\u00a0.0001). Thirteen (29.5%) patients received immune tolerance induction (ITI) therapy using low\u2010dose prothrombin complex concentrate regimens. Twelve completed ITI with three (25.0%) achieving success. Nephrotic syndrome developed in two (16.7%) patients during ITI.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study reports the largest Chinese cohort of HB inhibitor patients. Large deletions were most significantly associated with inhibitor development. Low\u2010dose ITI might be feasible for FIX inhibitor eradication.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14665","type":"journal-article","created":{"date-parts":[[2022,9,27]],"date-time":"2022-09-27T04:14:06Z","timestamp":1664252046000},"page":"123-134","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Factor IX inhibitors in haemophilia B: A report of National Haemophilia Registry in China"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3304-0079","authenticated-orcid":false,"given":"Xueqing","family":"Dou","sequence":"first","affiliation":[{"name":"State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology &amp; Blood Diseases Hospital, Chinese Academy of Medical Sciences &amp; Peking Union Medical College Tianjin Key Laboratory of Gene Therapy for Blood Diseases CAMS Key Laboratory of Gene Therapy for Blood Diseases  Tianjin China"},{"name":"National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology The First Affiliated Hospital of Soochow University  Suzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wenhui","family":"Zhang","sequence":"additional","affiliation":[{"name":"State Key Laboratory of Experimental Hematology National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology &amp; Blood Diseases Hospital, Chinese Academy of Medical Sciences &amp; Peking Union Medical College Tianjin Key Laboratory of Gene Therapy for Blood Diseases CAMS Key Laboratory of Gene Therapy for Blood Diseases  Tianjin China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7012-2420","authenticated-orcid":false,"given":"Man\u2010Chiu","family":"Poon","sequence":"additional","affiliation":[{"name":"Departments 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 Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2000-4087","authenticated-orcid":false,"given":"Linhua","family":"Yang","sequence":"additional","affiliation":[{"name":"Second Hospital of Shanxi Medical University  Taiyuan China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peng","family":"Cheng","sequence":"additional","affiliation":[{"name":"Department of Hematology Guangxi Medical University First Affiliated Hospital  Nanning China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shu","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Hematology The Second Affiliated Hospital of Chongqing Medical University  Chongqing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ying","family":"Wang","sequence":"additional","affiliation":[{"name":"Shenzhen Children's Hospital  Shenzhen 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