Prognostic scoring system for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children

A recent study suggests that a prognostic scoring system identified three high risk groups in children with statistically significant differences in survival curves based on the baseline characteristics that helped in risk prediction. The findings of this study were published in the journal, Pediatric Blood & Cancer.

This multicenter retrospective study included 264 children, aged 0-14 years who were diagnosed with Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH). The pediatric population was randomly divided into two groups: derivation (n=185) and verification (n=79). In order to establish a prognostic scoring system for death events, risk predictors were investigated using a Cox regression model during the follow-up period. Chronic active EBV infection (CAEBV) history, plasma EBV-DNA copy number, pulmonary infection hemorrhage of digestive tract, and hypoxemia prognostic scoring system (CEPHO-PSS) were developed which demarcated patients into low-risk (0-1 points), middle-risk (2-3 points), and high-risk (4-8 points) groups.

At the end of the study, the three risk groups showed survival curves with statistically significant differences. Receiver operating characteristic (ROC) and calibration curves in the derivation and verification cohorts, respectively, were used for internal and external verification of CEPHO-PSS, confirming good accuracy and applicability.

Thus, it can be concluded that the CEPHO-PSS may identify three risk groups in children with statistically significant differences in survival curves. This may help clinicians to check for risk prediction.