Prognostic scoring system for pediatric Epstein–Barr virus‐associated hemophagocytic lymphohistiocytosis based on baseline characteristics: A multicenter retrospective study

Abstract

AbstractBackgroundThe prognosis of pediatric Epstein–Barr virus (EBV)‐associated hemophagocytic lymphohistiocytosis (EBV‐HLH) varies. This study aimed to identify high‐risk children early.ProcedureData from 264 children (0–14 years of age), diagnosed with EBV‐HLH at six centers in China between January 2016 and December 2021, were analyzed. Patients were randomly divided into derivation (n = 185) and verification (n = 79) 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‐up period.ResultsChronic active EBV infection (CAEBV) history (hazard ratio [HR] 1.82 [95% confidence interval, CI: 1.02–3.26]; p = .0441), plasma EBV‐DNA more than 104 copies/mL (HR 2.89 [95% CI: 1.62–5.16]; p = .0003), pulmonary infection (HR 2.24 [95% CI: 1.06–4.75]; p = .0353), digestive tract hemorrhage (HR 2.55 [95% CI: 1.35–4.82]; p = .0041), and hypoxemia (HR 3.95 [95% CI: 2.15–7.26]; p < .0001) were independent risk factors. Accordingly, the CAEBV history, plasma EBV‐DNA copy number, pulmonary infection hemorrhage of digestive tract, hypoxemia prognostic scoring system (CEPHO‐PSS) were developed, which separated patients into low‐ (0–1 points), middle‐ (2–3 points), and high‐ (4–8 points) risk groups. Survival curves for the three groups exhibited statistically significant differences (p < .0001). Internal and external verification of CEPHO‐PSS was performed using receiver operating characteristic (ROC) and calibration curves in the derivation and verification cohorts, respectively, confirming good accuracy and applicability.ConclusionsThe CEPHO‐PSS 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.