目的报道1例以麻痹性肠梗阻为主要表现的噬血细胞综合征并相关文献复习。方法分析1例以麻痹性肠梗阻为主要表现的噬血细胞综合征临床资料及临床诊治经过。结果早期应用噬血细胞淋巴组织增生症(HLH)-2004方案,加强对症支持治疗,配合通腑泻热类中药灌肠,患者病情控制。结论噬血细胞综合征可累及多系统、多器官,包括肠道,及时诊断,早期足量应用HLH-2004方案,加强支持治疗,可能提高治疗成功率。 Objective To report a case of hemophagocytic syndrome with paralytic ileus as the main manifestation and related literature review. Methods One case of hemophagocytic syndrome with paralytic ileus as the main manifestation was reviewed and analyzed clinically. Results Early application of hemophagocytic lymphadenopathy (HLH) -2004 program to enhance symptomatic and supportive treatment, with Tongfu diarrhea enema enema, the patient’s condition control. Conclusion Hemophagocytic syndrome can be involved in multiple systems, multiple organs, including the intestine, timely diagnosis, early enough application of HLH-2004 program to enhance supportive care, may improve the success rate of treatment.