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Prognostic scoring system for pediatric Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis based on
Qi Kong1, Min Li2, Jingshi Wang1
1Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Insights
This study developed the CEPHO-PSS to identify high-risk children with Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH). The CEPHO-PSS aids clinicians in early risk stratification for improved patient outcomes.
Area of Science:
- Pediatric Hematology Oncology
- Infectious Diseases
- Immunology
Background:
- Prognosis of pediatric Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is variable.
- Early identification of high-risk pediatric EBV-HLH patients is crucial for timely intervention.
Purpose of the Study:
- To develop and validate a prognostic scoring system for early risk stratification of pediatric EBV-HLH.
- To identify independent risk factors associated with mortality in pediatric EBV-HLH.
Main Methods:
- Retrospective analysis of 264 pediatric EBV-HLH cases (0-14 years) from six Chinese centers (2016-2021).
- Patients were divided into derivation (n=185) and verification (n=79) cohorts.
- Cox regression analysis was used to identify risk predictors and develop the CEPHO-PSS.
Main Results:
- Independent risk factors identified: chronic active EBV infection history, plasma EBV-DNA >10^4 copies/mL, pulmonary infection, digestive tract hemorrhage, and hypoxemia.
- The developed CEPHO-PSS stratified patients into low, middle, and high-risk groups with significant survival differences (p < 0.0001).
- Internal and external validation confirmed the CEPHO-PSS's accuracy and applicability.
Conclusions:
- The CEPHO-PSS effectively identifies distinct risk groups in pediatric EBV-HLH patients.
- This scoring system, based on baseline characteristics, provides a convenient tool for clinical risk prediction.
- The CEPHO-PSS aids in early risk stratification, potentially improving management strategies for pediatric EBV-HLH.
Background:
The prognosis of pediatric Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH) varies. This study aimed to identify high-risk children early.
Procedure:
Data 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.
Results:
Chronic 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.
Conclusions:
The 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.
