{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T23:34:26Z","timestamp":1790033666371,"version":"4.0.1"},"reference-count":27,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,11,16]],"date-time":"2023-11-16T00:00:00Z","timestamp":1700092800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["82370185"],"award-info":[{"award-number":["82370185"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The prognosis of pediatric Epstein\u2013Barr virus (EBV)\u2010associated hemophagocytic lymphohistiocytosis (EBV\u2010HLH) varies. This study aimed to identify high\u2010risk children early.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>Data from 264 children (0\u201314\u00a0years of age), diagnosed with EBV\u2010HLH at six centers in China between January 2016 and December 2021, were analyzed. Patients were randomly divided into derivation (<jats:italic>n<\/jats:italic>\u00a0=\u00a0185) and verification (<jats:italic>n<\/jats:italic>\u00a0=\u00a079) cohorts. A Cox regression model was used to explore risk predictors and establish a prognostic scoring system for death events that occurred during the follow\u2010up period.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Chronic active EBV infection (CAEBV) history (hazard ratio [HR] 1.82 [95% confidence interval, CI: 1.02\u20133.26]; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.0441), plasma EBV\u2010DNA more than 10<jats:sup>4<\/jats:sup>\u00a0copies\/mL (HR 2.89 [95% CI: 1.62\u20135.16]; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.0003), pulmonary infection (HR 2.24 [95% CI: 1.06\u20134.75]; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.0353), digestive tract hemorrhage (HR 2.55 [95% CI: 1.35\u20134.82]; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.0041), and hypoxemia (HR 3.95 [95% CI: 2.15\u20137.26]; <jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.0001) were independent risk factors. Accordingly, the CAEBV history, plasma EBV\u2010DNA copy number, pulmonary infection hemorrhage of digestive tract, hypoxemia prognostic scoring system (CEPHO\u2010PSS) were developed, which separated patients into low\u2010 (0\u20131 points), middle\u2010 (2\u20133 points), and high\u2010 (4\u20138 points) risk groups. Survival curves for the three groups exhibited statistically significant differences (<jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.0001). Internal and external verification of CEPHO\u2010PSS was performed using receiver operating characteristic (ROC) and calibration curves in the derivation and verification cohorts, respectively, confirming good accuracy and applicability.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The CEPHO\u2010PSS identified three risk groups with statistically significant differences in survival curves. It was based on the baseline characteristics, and can give clinicians a convenient check for risk prediction.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.30772","type":"journal-article","created":{"date-parts":[[2023,11,17]],"date-time":"2023-11-17T08:36:06Z","timestamp":1700210166000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Prognostic scoring system for pediatric Epstein\u2013Barr virus\u2010associated hemophagocytic lymphohistiocytosis based on baseline characteristics: A multicenter retrospective study"],"prefix":"10.1002","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7077-8905","authenticated-orcid":false,"given":"Qi","family":"Kong","sequence":"first","affiliation":[{"name":"Department of Hematology Beijing Friendship Hospital Capital Medical University  Beijing 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Center Sun Yat\u2010Sen Memorial Hospital Sun Yat\u2010Sen University  Guangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minghua","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Third Xiangya Hospital Central South University  Changsha Hunan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaojun","family":"Xu","sequence":"additional","affiliation":[{"name":"Department of Hematology\u2010Oncology The Children's Hospital of Zhejiang University School of Medicine  Hangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5001-7933","authenticated-orcid":false,"given":"Zhen","family":"Tan","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\u2010Oncology Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine  Shanghai 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