目的　探讨儿童噬血细胞综合征(HLH)的临床特点及诊治策略。方法　回顾性分析2009至2013年住院治疗的11例HLH患儿的临床资料。结果　11例患儿中，6例为EB病毒感染相关性HLH，1例为T细胞淋巴瘤相关性HLH，2例病因不明，2例为UNC13D基因编码序列突变，分别为c.2459C>T/p.A832V(丙氨酸突变为缬氨酸)，c.3067C>T/p.R1023C(精氨酸突变为半胱氨酸)；11例患儿中6例经治疗后病情好转，5例治疗无效死亡。结论　儿童HLH临床表现缺乏特异性，病情发展快，须及时诊断、治疗。

　Objectives　To explore the clinical presentation, diagnosis and treatment of hemophagocytic syndrome (HLH) in children. Methods　The clinical data from 11 hospitalized pediatric patients with HLH were collected and retrospectively analyzed from 2009 to 2013. Results　In 11 pediatric patients with HLH, 6 patients were Epstein-Barr virus associated-HLH, 1 patient was T-cell lymphoma associated-HLH, 2 patients had unknown reason, 2 patients had mutations in the UNC13D gene coding sequence, c.2459C>T/p.A832V (alanine to valine mutation) and c.3067C>T/p.R1023C (arginine to cysteine mutation) respectively. In 11 patients, 6 patients were improved after treatment and 5 patients were died. Conclusions 　The HLH in children lacks specific clinical presentation and progresses rapidly. It should be diagnosed and treated in time.