Prognostic factors of early death in children with hemophagocytic lymphohistiocytosis

• Published in: Cytokine (Philadelphia, Pa.) Vol. 97; pp. 80 - 85
• Main Authors: Luo, Ze-Bin, Chen, Yuan-Yuan, Xu, Xiao-Jun, Zhao, Ning, Tang, Yong-Min
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.09.2017
• Subjects: Child; Child, Preschool; Children; Cytokine; Early death; Female; Hemophagocytic lymphohistiocytosis; Humans; Infant; Infant, Newborn; Interferon-gamma - blood; Interleukin-10 - blood; Interleukin-6 - blood; Liver - pathology; Liver damage; Lymphohistiocytosis, Hemophagocytic - diagnosis; Lymphohistiocytosis, Hemophagocytic - metabolism; Lymphohistiocytosis, Hemophagocytic - mortality; Male; Multivariate Analysis; Prognosis; Proportional Hazards Models; Retrospective Studies; Risk Factors; AST; CBA; IL; HLH; Cytokine; Liver damage; ALT; Hemophagocytic lymphohistiocytosis; LDH; IFN-γ; TNF-α; ANC; Tbil; EBV; ALC; Early death; Children
• ISSN: 1043-4666; 1096-0023
• DOI: 10.1016/j.cyto.2017.03.013

•IL-10, IFN-γ and IL-6 levels are higher elevated in HLH patients with early death.•IL-10, albumin and LDH levels at diagnosis can predict early death for HLH patients.•The number of risk factors at diagnosis can identify patients with high risk for early death. Hemophagocytic lymphohistiocytosis is a rapidly progressing and fatal disease. Early identification of early death for HLH patients based on the laboratory findings at the time of diagnosis could improve the overall survival. A retrospective study was performed on 95 Chinese pediatric patients with HLH. Patients’ data including clinical features and laboratory findings at diagnosis were collected. In a multivariate Cox proportional hazard regression model analysis, albumin≤27.75g/L (hazard ratio (HR)=11.82, 95% confidence interval (CI) 2.58–54.23; P=0.001), LDH≥3707.5 U/L (HR=4.15, 95%CI 1.43–12.01; P=0.009), and IL-10≥456pg/ml (HR=12.39, 95%CI 1.59–96.79; P=0.016) at diagnosis were independent prognostic factors of early death. The risk of early death was 33-fold increase in patients with three risk factors (HR=33.33; 95%CI 8.40–125.00; P<0.001), and 12-fold increase in patients with two risk factors (HR=12.80; 95%CI 2.34–69.80; P=0.002) when compared to it in patients with zero to one risk factor. Our results reveal that HLH patients with the risk of early death can be identified by laboratory findings at diagnosis, which may help guide the treatment decision making for this disease.