目的 分析儿童噬血细胞综合征（HPS）的临床特点及预后影响因素。方法 回顾性分析2017年1月至2023年7月收治的45例HPS患儿的临床资料及随访数据。结果 45例患儿中位发病年龄为4.5（2.1~10.0）岁，均以发热起病，感染相关HPS占77.8%，其中EB病毒（EBV）感染最多（85.7%）。所有患儿总生存率（88.9±4.7）%。单因素分析显示三酰甘油≥3.8 mmol/L、乳酸脱氢酶≥2 500 U/L是影响患儿预后的危险因素（P均<0.05），多因素分析显示三酰甘油≥3.8 mmol/L是影响患儿预后的独立危险因素（P=0.029）。结论 HPS常以发热起病，感染（尤其是EBV感染）为最常见的病因；初诊时三酰甘油及乳酸脱氢酶水平（尤其是三酰甘油≥3.8 mmol/L）是评估预后的关键因素。

Objective To analyze the clinical characteristics and prognostic factors of hemophagocytic syndrome (HPS) in children. Methods A retrospective analysis was conducted on the clinical data and follow-up information of 45 children with HPS admitted from January 2017 to July 2023. Results The median age of onset in the 45 children was 4.5 (2.1-10.0) years, all of whom started with fever. Infection-related HPS accounted for 77.8%, with Epstein-Barr virus (EBV) infection being the most common (85.7%). The overall survival rate of all children was (88.9±4.7)%. Univariate analysis showed that triglycerides≥3.8 mmol/L and lactate dehydrogenase≥2500 U/L were risk factors affecting the prognosis of children with HPS (P<0.05 for both), and multivariate analysis showed that triglycerides≥3.8 mmol/L was an independent risk factor affecting the prognosis of children with HPS (P=0.029). Conclusion HPS often starts with fever, and infection (especially EBV infection) is the most common cause; the levels of triglycerides and lactate dehydrogenase at initial diagnosis (especially triglycerides≥3.8 mmol/L) are key factors in assessing prognosis.