目的：提高对以病毒性脑炎起病的噬血细胞综合征的临床表现、诊断标准及治疗方案的认识。

方法：报道1例神经内科收治的以病毒性脑炎症状起病的噬血细胞综合征病例的诊疗经过，结合文献复习，讨论噬血细胞综合征的病因、分类、临床表现、诊断标准和治疗措施。

结果：1例以发热和头痛为主诉的青年患者的病情进展迅速，出现感染性休克和弥散性血管内凝血，经实验室检查及多学科会诊，诊断为噬血细胞综合征，给予地塞米松、丙种球蛋白、抗感染和输注血小板等治疗后，痊愈出院。

结论：噬血细胞综合征是一种免疫调节异常导致的过度炎性反应综合征，可发生在各年龄段，可危及生命。提高对噬血细胞综合征的临床表现、诊断标准和治疗措施的认识，有助于尽早诊断，并给予合适的治疗。

Objective: To improve the understanding of clinical manifestations, diagnostic criteria and therapeutic measures of hemophagocytic lymphohistiocytosis (HLH) with viral encephalitis onset.

Methods: This paper reports the diagnosis and treatment of a case diagnosed as hemophagocytic lymphohistiocytosis complicated with viral encephalitis symptoms onset admitted to the department of neurology. Based on literature review, the etiology, classification, clinical manifestations, diagnostic criteria and treatment of hemophagocytic syndrome were discussed.

Results: One young patient complained of fever and headache, and his condition progressed rapidly, resulting in infectious shock and disseminated intravascular coagulation. After laboratory examination and multidisciplinary discussion, the patient was diagnosed as hemophagocytic lymphohistiocytosis. Dexamethasone, gamma globulin, anti-infection, platelet transfusion and other treatments were given, and the patient was discharged with fully recovery.

Conclusion: Hemophagocytic lymphohistiocytosis is an overactive inflammatory response syndrome caused by abnormal immune regulation that can occur at all ages, and it can be life-threatening. To improve the understanding of the clinical manifestations, diagnostic criteria and treatment measures of HLH is helpful for early diagnosis and appropriate treatment.