目的:探讨皮下脂膜炎样T细胞淋巴瘤(SPTCL)合并噬血细胞综合征(HPS)的诊断及治疗。方法:报道2例SPTCL合并HPS患者并进行文献复习。结果:2例患者治疗过程中HPS反复发作,经多次病理检查最终确诊为SPTCL,给予原发病治疗后长期生存。结论:SPTCL合并HPS少见,原发病诊断困难,病情反复。尽早且多次进行淋巴结、骨髓或病变部位组织病理检查以寻找原发病,给予原发病治疗,是控制SPTCL合并HPS的关键。 Objective: To investigate the diagnosis and treatment of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) combined with hemophagocytic syndrome (HPS). Methods: Two cases of SPTCL with HPS were reported and reviewed. Results: HPS was recurred in 2 patients during the course of treatment. After multiple pathological examinations, the patients were finally diagnosed as SPTCL, and the long-term survival was given after the primary disease was treated. Conclusion: The combination of SPTCL and HPS is rare. The primary disease is difficult to diagnose and the condition is repeated. Early and repeated lymph node, bone marrow or lesion histopathological examination to find the primary disease, given the primary disease treatment, is the key to control SPTCL combined HPS.