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Prognostic factors of early outcome in pediatric hemophagocytic lymphohistiocytosis: an analysis of 116 cases

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Abstract

Early mortality remains a major challenge for the treatment of hemophagocytic lymphohistiocytosis (HLH), which warrants the need for prompt risk stratification in the early phase of the disease. We retrospectively analyzed clinical features of a cohort of pediatric patients managed at a tertiary hospital in southern China from 2005 to 2015. A total of 116 patients (median age 27.5 months) with predominantly secondary HLH were included. In a multivariate Cox regression model, neutrophils <0.5 × 109/L (risk ratio (RR) = 5.01; 95 % confidence interval (CI) 1.55–16.20; P = 0.007), total bilirubin over twofold upper limit of normal value (RR = 2.86; 95 % CI 0.83–9.88; P = 0.097), and albumin ≤20 g/L (RR = 5.79; 95 % CI 1.70–19.73; P = 0.005) at diagnosis were independent risk factors for 30-day mortality. The 30-day overall survival rate (OS) of patients with three risk factors was significantly lower than that of patients with zero to two risk factors (0 vs 90.7 %; P<0.001). Patients with three risk factors were 64-fold more likely to have early adverse outcome as compared to patients with zero to two risk factors (RR = 64.45; 95 % CI 18.35–226.33; P<0.001). Platelet count normalization in 2 weeks was an independent predictor for resolution after initial therapy with an odds ratio (OR) of 18.4 (95 % CI 2.7–122.9; P = 0.003). Our results indicate that severe neutropenia and liver function damage are prognostic factors for early death in HLH and platelet count normalization in 2 weeks is a critical predictor for resolution after initial therapy.

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Acknowledgments

This work was supported by grants provided by the National Natural Science Foundation of China (NO. 30860308; NO. 81160070). We thank all of the patients who gave consent to participate in this study.

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  1. Department of Pediatrics, The First Affiliated Hospital of Guangxi Medical University, Guangxi Medical University, Nanning, 530021, Guangxi, China

    Qiong Bin, Jin-Hong Gao & Jian-Ming Luo

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  1. Qiong Bin
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Correspondence to Jian-Ming Luo.

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Approval to perform this study was given by the First Affiliated Hospital of Guangxi Medical University Institutional Review Board. Informed consent was obtained from all patients for being included in the study.

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The authors declare that they have no conflict of interest.

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Bin, Q., Gao, JH. & Luo, JM. Prognostic factors of early outcome in pediatric hemophagocytic lymphohistiocytosis: an analysis of 116 cases. Ann Hematol 95, 1411–1418 (2016). https://doi.org/10.1007/s00277-016-2727-6

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