{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T20:13:31Z","timestamp":1783455211479,"version":"3.55.0"},"reference-count":25,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,5,10]],"date-time":"2026-05-10T00:00:00Z","timestamp":1778371200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,5,10]],"date-time":"2026-05-10T00:00:00Z","timestamp":1778371200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["22ek0109612h0001"],"award-info":[{"award-number":["22ek0109612h0001"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["23ek0109612h0002"],"award-info":[{"award-number":["23ek0109612h0002"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["24ek0109612h0003"],"award-info":[{"award-number":["24ek0109612h0003"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["25ek0109807h0001"],"award-info":[{"award-number":["25ek0109807h0001"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["24FC1001"],"award-info":[{"award-number":["24FC1001"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["26FC1002"],"award-info":[{"award-number":["26FC1002"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    Systemic chronic active Epstein\u2013Barr virus disease (sCAEBV) is a life\u2010threatening disorder characterized by Epstein\u2013Barr virus (EBV) infection in T cells and\/or natural killer (NK) cells leading to systemic inflammation. We retrospectively analysed 46 adult patients diagnosed with sCAEBV at two high\u2010volume centres in Japan. Patients with active disease received immunosuppressive therapy, chemotherapy\u2010based regimens and\/or ruxolitinib. Allogeneic haematopoietic stem cell transplantation (allo\u2010HSCT) was pursued whenever feasible. Of 46 patients, 41 (89.1%) ultimately underwent allo\u2010HSCT. Five patients progressed from hydroa vacciniforme lymphoproliferative disorder (HV\u2010LPD); all showed EBV\u2010infected CD4+ cells. The 3\u2010year overall survival (OS) was 43.1% (95% confidence interval [CI], 27.7\u201357.5), and all patients who did not undergo allo\u2010HSCT died within 8\u2009months from diagnosis. Five patients (10.9%) developed lymphoma or leukaemia. Leading causes of death included haemophagocytic lymphohistiocytosis and infections. Multivariable analysis identified high alanine aminotransferase (ALT) and ferritin as independent predictors of poor outcome. Based on these findings, we developed a risk model stratifying patients into three groups with distinct 3\u2010year OS rates: 69.9% (95% CI, 44.6\u201385.3) in patients with neither factor, 24.8% (95% CI, 7.0\u201348.2) in those with one factor and 0% in those with both factors (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001). ALT and ferritin are practical biomarkers for risk stratification and treatment.\n                  <\/jats:p>","DOI":"10.1111\/bjh.70532","type":"journal-article","created":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T04:54:22Z","timestamp":1778475262000},"page":"96-106","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical features and prognostic factors of adult systemic chronic active\n                    <scp>EBV<\/scp>\n                    disease: A retrospective analysis in Japan"],"prefix":"10.1111","volume":"209","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-5004-3096","authenticated-orcid":false,"given":"Aika","family":"Fuseya","sequence":"first","affiliation":[{"name":"Department of Hematology and Oncology St. Marianna University School of Medicine  Kawasaki Kanagawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3142-8806","authenticated-orcid":false,"given":"Masahide","family":"Yamamoto","sequence":"additional","affiliation":[{"name":"Department of Hematology, Graduate School of Medical and Dental Sciences Institute of Science Tokyo  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2465-2663","authenticated-orcid":false,"given":"Miwako","family":"Nishio","sequence":"additional","affiliation":[{"name":"Department of Hematology and Biophysical Systems Analysis, Graduate School of Medical and Dental Sciences Institute of Science Tokyo  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5807-0659","authenticated-orcid":false,"given":"Ryo","family":"Kitabayashi","sequence":"additional","affiliation":[{"name":"Department of Clinical Biostatistics, Graduate School of Medical and Dental Sciences Institute of Science Tokyo  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2580-7460","authenticated-orcid":false,"given":"Akihiro","family":"Hirakawa","sequence":"additional","affiliation":[{"name":"Department of Clinical Biostatistics, Graduate School of Medical and Dental Sciences Institute of Science Tokyo  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0905-6855","authenticated-orcid":false,"given":"Hiroshi","family":"Yasui","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology St. Marianna University School of Medicine  Kawasaki Kanagawa Japan"},{"name":"Institute of Medical Science The University of Tokyo  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1027-1552","authenticated-orcid":false,"given":"Yoshihiro","family":"Umezawa","sequence":"additional","affiliation":[{"name":"Department of Hematology, Graduate School of Medical and Dental Sciences Institute of Science Tokyo  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-2716-3377","authenticated-orcid":false,"given":"Keisuke","family":"Tanaka","sequence":"additional","affiliation":[{"name":"Department of Hematology, Graduate School of Medical and Dental Sciences Institute of Science Tokyo  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9652-4559","authenticated-orcid":false,"given":"Hayato","family":"Tamai","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology St. Marianna University School of Medicine  Kawasaki Kanagawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8422-2959","authenticated-orcid":false,"given":"Yu","family":"Uemura","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology St. Marianna University School of Medicine  Kawasaki Kanagawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6164-4796","authenticated-orcid":false,"given":"Kumi","family":"Oshima","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology St. Marianna University School of Medicine  Kawasaki Kanagawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fumiaki","family":"Sano","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology St. Marianna University School of Medicine  Kawasaki Kanagawa 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