Prognostic Factors of Early Death in Childhood Hemophagocytic Lymphohistiocytosis: Experience From A Single Tertiary Center in Thailand

Abstract

Objective: To establish the clinical profile, outcomes, and risk factors of mortality in pediatric hemophagocytic lymphohistiocytosis (HLH) patients admitted to a tertiary care hospital in the south of Thailand. Material and Methods: The medical records of HLH patients aged under 15 years were retrospectively reviewed. Survival times were estimated using the Kaplan-Meier estimator. Univariate associations between covariates and survival were visualized with graphs and compared using the log-rank test. Factors with statistical significance in the univariate analysis were included in a multivariate cox regression analysis. Results: A total of 24 childhood HLH cases were identified over the 20-year study period. Central nervous system (CNS) involvement at diagnosis (hazard ratio [HR]: 7.7, 95% CI: 1.2-51.2), absolute neutrophil count (ANC) <1,000.0 cells/μL (HR: 33.3, 95% CI: 2.7-39.8) and renal insufficiency (HR: 28.2, 95% CI: 2.1-373.8) were adverse risk factors. Forty-six percent of the children survived at least 30 days after diagnosis with a median survival time of 21 days. Conclusion: CNS involvement, low ANC and renal insufficiency at diagnosis were adverse risk factors for early death in HLH children.

Keywords

• hemophagocytic lymphohistiocytosis
• prognostic factors
• survival outcome