Development and validation of a risk stratification score for hemophagocytic syndrome in chronic active Epstein-Barr virus infection: A 10-year retrospective cohort study
Year: 2025
Type: article
Study design: Observational Study
Abstract: OBJECTIVE: This study aims to identify risk factors for the development of hemophagocytic lymphohistiocytosis (HLH) within three years among patients with chronic active Epstein-Barr virus infection (CAEBV) and to construct a risk stratification score. METHODS: A retrospective cohort study was conducted involving 165 patients with CAEBV admitted to two infectious disease centers in Northwest China between January 2012 and December 2022, through which we identified independent risk factors for HLH. Cox regression was utilized to establish the nomogram model and generate the risk stratification score. The discrimination and clinical utility of both the nomogram and score models were subsequently assessed using calibration plots, receiver operating characteristic (ROC) curves, decision curve analysis (DCA), and cross-validation. RESULTS: Among the 165 patients, 98 (59.4 %) developed HLH within three years. The median progression time was 405 days (IQR: 217, 740), with cumulative incidence rates of HLH at 1, 2, and 3 years of 41.3 %, 56.1 %, and 65.9 %, respectively. Five independent predictors were identified: cytopenia (HR: 1.70, 95 % CI: 1.07-2.70), EBV-DNA load in plasma (HR: 2.02, 95 % CI: 1.46-2.80), alanine aminotransferase (HR: 1.34, 95 % CI: 1.12-1.60), ferritin (HR: 1.01, 95 % CI: 1.01-1.01), and triglycerides (HR: 1.21, 95 % CI: 1.05-1.38). Both the nomogram and simplified score model demonstrated excellent discrimination (AUC > 0.7) and reliable calibration within a 3-year timeframe. The score model exhibited predictive power comparable to the nomogram, as indicated by net reclassification index and integrated discrimination index analyses. CONCLUSION: The validated nomogram and score model facilitate rapid HLH risk stratification in CAEBV patients, supporting clinical risk assessment and early intervention.
Authors: Fei Hu, Jiayi Zhan, Meijuan Peng, Haifeng Hu, Xuyang Zheng, Danfeng Ren, Rui Li, Hong Du, Wen Tian, Zhanhu Bi, Denghui Yu, Hongkai Xu, Xiaofei Yang, Yan Liang, Fangfang Li, Jingtao Hou, Yingli He, Jianqi Lian
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,310
Award: G1010520453
OpenAlex ID: W4416427057
