患儿，女性，10岁，因发热5 d，发现外周血三系减少2 d入院。骨髓检查显示存在吞噬现象，外周血检验提示三系减少、铁蛋白升高、纤维蛋白原降低、甘油三酯升高、可溶性CD25升高及自然杀伤细胞活性降低，考虑为噬血细胞综合征。入院当天，患儿出现抽搐，并迅速进展为难治性癫痫持续状态，符合热性感染相关性癫痫综合征。经积极免疫治疗，患儿噬血细胞综合征得到控制，但遗留难治性癫痫，认知水平基本正常。该例由噬血细胞综合征导致热性感染相关性癫痫综合征的情况，为国内首次报道，旨在提升临床医生对该类疾病的认识。

The patient was a girl, aged 10 years, who was admitted due to fever for 5 days and pancytopenia in peripheral blood for 2 days. Bone marrow examination showed the presence of phagocytic activity, and peripheral blood tests showed pancytopenia, an increase in ferritin, a reduction in fibrinogen, increases in triglyceride and sCD25, and a reduction in natural killer cell activity, which led to the diagnosis of hemophagocytic lymphohistiocytosis (HLH). On the day of admission, the child developed convulsions and rapidly progressed to refractory status epilepticus, which was consistent with the manifestations of febrile infection-related epilepsy syndrome. HLH was controlled after active immunotherapy, with the sequela of refractory epilepsy, and her cognitive function was essentially within normal limits. This article reports the condition of febrile infection-related epilepsy syndrome caused by HLH for the first time in China, in order to improve the awareness of this disease among clinicians.