{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T17:22:19Z","timestamp":1767979339598,"version":"3.49.0"},"reference-count":34,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,12,8]],"date-time":"2022-12-08T00:00:00Z","timestamp":1670457600000},"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":[[2023,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Autoimmune factor X (FX or F10) deficiency (AiF10D) is an extremely rare acquired haemorrhagic disorder characterized by a severe reduction in FX activity due to autoantibodies against FX.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>Anti\u2010FX autoantibodies were investigated in four patients with suspected AiF10D, and their properties were analysed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>Anti\u2010FX auto antibodies in plasma were detected by ELISA with three of four cases. One case of anti\u2010FX autoantibody negativity was later diagnosed as AL\u2010amyloidosis. IgG1 and IgG3 coexisted in all anti\u2010FX autoantibodies of the three patients with AiF10D (cases X1, X2, and X3). Western blot analysis showed that the antibodies were bound to the FX light chain for cases X2 and X3, but the binding was weak for case X1. When the fusion proteins of a secretory luciferase with full\u2010length FX or its \u03b3\u2010carboxylated glutamic acid (Gla) domain were added to the plasma of the three patients, both fusion proteins were immunoprecipitated as antigen\u2010antibody complexes. Contrarily, the latter fusion protein produced in the presence of warfarin demonstrated a decrease in the collection rate, suggesting that their autoantibodies recognized the light chain and regions containing Gla residues. Since all three patients were essentially negative for FX inhibitors, it was concluded that the anti\u2010FX autoantibodies for these cases were predominantly non\u2010neutralizing. The concentration of the FX antigen also significantly reduced in these patients, suggesting that anti\u2010FX autoantibodies promote the clearance of FX.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Immunological anti\u2010FX autoantibody detection is highly recommended to ensure that AiF10D cases are not overlooked, and to start necessary immunosuppressive therapies.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14711","type":"journal-article","created":{"date-parts":[[2022,12,8]],"date-time":"2022-12-08T14:36:10Z","timestamp":1670510170000},"page":"555-563","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Identification of non\u2010neutralizing anti\u2010factor X autoantibodies in three Japanese cases of autoimmune acquired factor X deficiency"],"prefix":"10.1111","volume":"29","author":[{"given":"Masayoshi","family":"Souri","sequence":"first","affiliation":[{"name":"Department of Molecular Patho\u2010Biochemistry and Patho\u2010Biology Yamagata University School of Medicine  Yamagata Japan"},{"name":"Japanese Collaborative Research Group on Autoimmune Coagulation Factor Deficiency supported by the Japanese Ministry of Health Labor and Welfare  Yamagata Japan"},{"name":"Department of Public Health and Hygiene Yamagata University School of Medicine  Yamagata Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tsukasa","family":"Osaki","sequence":"additional","affiliation":[{"name":"Department of Molecular Patho\u2010Biochemistry and Patho\u2010Biology Yamagata University School of Medicine  Yamagata Japan"},{"name":"Japanese Collaborative Research Group on Autoimmune Coagulation Factor Deficiency supported by the Japanese Ministry of Health Labor and Welfare  Yamagata Japan"},{"name":"Department of Public Health and Hygiene Yamagata University School of Medicine  Yamagata Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuji","family":"Shimura","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology Department of Medicine Kyoto Prefectural University of Medicine  Kyoto Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Ichikawa","sequence":"additional","affiliation":[{"name":"Department of Hematology Tohoku University Hospital  Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makiko","family":"Mori","sequence":"additional","affiliation":[{"name":"Department of Clinical Laboratory Toyama Prefectural Central Hospital  Toyama Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiyuki","family":"Ogawa","sequence":"additional","affiliation":[{"name":"Japanese Collaborative Research Group on Autoimmune Coagulation Factor Deficiency supported by the Japanese Ministry of Health Labor and Welfare  Yamagata 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SasadaY KunikadoR et\u00a0al.Experience with administration of cryoprecipitate to an elderly patient with undetermined type of bleeding tendency. 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