{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T08:59:59Z","timestamp":1780909199519,"version":"3.54.1"},"reference-count":21,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,3,29]],"date-time":"2026-03-29T00:00:00Z","timestamp":1774742400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,3,29]],"date-time":"2026-03-29T00:00:00Z","timestamp":1774742400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82370189"],"award-info":[{"award-number":["82370189"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012166","name":"National Key Research and Development Program of China","doi-asserted-by":"publisher","award":["2024YFC2309803"],"award-info":[{"award-number":["2024YFC2309803"]}],"id":[{"id":"10.13039\/501100012166","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012166","name":"National Key Research and Development Program of China","doi-asserted-by":"publisher","award":["2023YFC2706100"],"award-info":[{"award-number":["2023YFC2706100"]}],"id":[{"id":"10.13039\/501100012166","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":[[2026,5]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    Chronic active Epstein\u2013Barr virus disease (CAEBV), an Epstein\u2013Barr virus (EBV)+ T\/Natural Killer (NK)\u2010cell lymphoproliferative disorder, remains critically undefined in treatment and prognostic stratification due to its rarity. We analysed 149 paediatric systemic CAEBV patients, of whom 134 received LDEP (liposomal doxorubicin, etoposide, pegylated asparaginase and methylprednisolone) chemotherapy as a bridge to haematopoietic stem cell transplantation (HSCT). Diagnostic delay was common (median 6.0\u2009months), with 41.6% presenting atypically with localized symptoms at onset. Haemophagocytic lymphohistiocytosis developed in 50.3%. EBV infection involved pan\u2010lymphoid lineages in 85.2%, with NK\u2010cell predominance (44.3%) enriched in the hydroa vacciniforme\/severe mosquito bite allergy subtype. LDEP induced favourable response in 59.0%, significantly improving HSCT rates (98.7% vs. 69.1%). Overall, 127 patients (85.2%) underwent HSCT, with 3\u2010year post\u2010transplant survival of 84.5%\u2009\u00b1\u20093.5%. Inferior post\u2010HSCT survival was independently associated with active disease at transplantation (hazard ratio [HR]\u2009=\u20096.17,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001) and also linked to longer chemotherapy\u2010to\u2010HSCT interval (&gt;3.5\u2009months) and poor LDEP response. Non\u2010transplanted patient had 90.9% mortality. The entire cohort had a 3\u2010year overall survival of 72.6% from diagnosis, with poor LDEP response, hepatic dysfunction and leucopenia as independent risk factors. This large paediatric cohort defines the clinical spectrum of CAEBV, establishes LDEP as an effective bridging regimen to HSCT\u2014particularly in early\u2010stage disease\u2014and highlights the need for early disease activity control and timely transplantation.\n                  <\/jats:p>","DOI":"10.1111\/bjh.70454","type":"journal-article","created":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T00:34:46Z","timestamp":1774830886000},"page":"1704-1712","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Chronic active Epstein\u2013Barr virus disease in children: Clinical outcomes and prognostic risk factors from a 149\u2010patient cohort"],"prefix":"10.1111","volume":"208","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0404-5743","authenticated-orcid":false,"given":"Qing","family":"Zhang","sequence":"first","affiliation":[{"name":"Hematologic Disease Laboratory Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"},{"name":"National Key Discipline of Pediatrics Capital Medical University  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5476-4677","authenticated-orcid":false,"given":"Yun\u2010Ze","family":"Zhao","sequence":"additional","affiliation":[{"name":"National Key Discipline of Pediatrics Capital Medical University  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing China"},{"name":"Department of Hematology, Hematology Center Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dong","family":"Wang","sequence":"additional","affiliation":[{"name":"National Key Discipline of Pediatrics Capital Medical University  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing China"},{"name":"Department of Hematology, Hematology Center Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong\u2010Hao","family":"Ma","sequence":"additional","affiliation":[{"name":"National Key Discipline of Pediatrics Capital Medical University  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing China"},{"name":"Department of Hematology, Hematology Center Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lei","family":"Cui","sequence":"additional","affiliation":[{"name":"Hematologic Disease Laboratory Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"},{"name":"National Key Discipline of Pediatrics Capital Medical University  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei\u2010Jing","family":"Li","sequence":"additional","affiliation":[{"name":"Hematologic Disease Laboratory Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"},{"name":"National Key Discipline of Pediatrics Capital Medical University  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chan\u2010Juan","family":"Wang","sequence":"additional","affiliation":[{"name":"National Key Discipline of Pediatrics Capital Medical University  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing China"},{"name":"Department of Hematology, Hematology Center Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhi\u2010Gang","family":"Li","sequence":"additional","affiliation":[{"name":"Hematologic Disease Laboratory Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"},{"name":"National Key Discipline of Pediatrics Capital Medical University  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3727-1192","authenticated-orcid":false,"given":"Rui","family":"Zhang","sequence":"additional","affiliation":[{"name":"National Key Discipline of Pediatrics Capital Medical University  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing China"},{"name":"Department of Hematology, Hematology Center Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing 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