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This retrospective study analysed the efficacy and safety of RUX\u2010based regimen (<jats:italic>n<\/jats:italic>\u2009=\u200953) and compared it with adjusted HLH\u201094 chemotherapy (<jats:italic>n<\/jats:italic>\u2009=\u200942) in the treatment of paediatric EBV\u2010HLH. The patients treated with the RUX\u2010based regimen received RUX monotherapy as front\u2010line therapy. Additional methylprednisolone and etoposide would be added in sequence if the response was unsatisfactory. At 8\u2009weeks of therapy, the overall response rate of the RUX group was comparable to that of the traditional chemotherapy group (84.9% vs. 76.2%, <jats:italic>p<\/jats:italic>\u2009=\u20090.282), with a 12\u2010month survival rate of 92.2% (95% CI: 80.6\u201397.0) and 87.6% (95% CI: 72.1\u201394.5) (<jats:italic>p<\/jats:italic>\u2009=\u20090.595). In the RUX group, 56.6% of patients achieved sustained remission without requiring etoposide during a median follow\u2010up of 17.2\u2009months. Among these patients, 93.3% had primary EBV infection. The RUX\u2010based regimen was well\u2010tolerated, exhibiting significantly lower incidence of myelosuppression and secondary infection than adjusted HLH\u201094 chemotherapy (35.8% vs. 95.2%, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001; 28.3% vs. 59.5%, <jats:italic>p<\/jats:italic>\u2009=\u20090.002). Overall, our preliminary results indicated that EBV\u2010HLH children treated with the RUX\u2010based regimen demonstrated favourable efficacy while significantly reducing treatment\u2010related adverse events.<\/jats:p>","DOI":"10.1111\/bjh.20264","type":"journal-article","created":{"date-parts":[[2025,7,16]],"date-time":"2025-07-16T03:44:11Z","timestamp":1752637451000},"page":"902-910","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Efficacy and safety of ruxolitinib\u2010based regimen in the treatment of paediatric Epstein\u2013Barr virus\u2010associated haemophagocytic lymphohistiocytosis"],"prefix":"10.1111","volume":"207","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2057-4920","authenticated-orcid":false,"given":"Wenqian","family":"Wang","sequence":"first","affiliation":[{"name":"Hematology Center, Beijing Children's Hospital Capital Medical University, National Center for Children's Health  Beijing 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