Objective To explore the clinical features and treatment regimen for patients with subcutaneous panniculitis - like T - cell lymphoma associated with secondary hemophagocytic syndrome. Methods The clinical manifestations and related laboratory examinations,the duration for diagnosis of the disease,treatment and prognosis of 6 patients were analyzed and compared. Results All these 6 patients were diag-nosed as hemophagocytic syndrome,. they had fever,splenomegaly,elevated serum level of ferritin,hemophagocytosis and abnormal liver function. It had spent 5 to 18 months for the diagnosis of hemophagocytic syndrome to final diagnosis of lymphoma. One patient gave up the treatment and died,and the remaining patients received chemotherapy based on CHOP regimen. One patient received autologous hematopoietic stem cell trans-plantation,and 2 patients received allogeneic hematopoietic stem cell transplantation. One patient died of graft - versus - host disease(GVHD), and the conditions of remaining patients were stable during the follow - up period. Conclusion Subcutaneous panniculitis - like T - cell lympho-ma associated with hemophagocytic syndrome is rare. In order to determine the primary disease,it should actively control the symptoms of hemoph-agocytic syndrome. The progress of the disease is rapid,and the application of high dosage chemotherapy combined with autologous hematopoietic stem cell transplantation or allogeneic hematopoietic stem cell transplantation should be considered.%目的：探讨皮下脂膜炎样 T 细胞淋巴瘤继发噬血细胞综合征的临床特点与治疗。方法对6例皮下脂膜炎样 T 细胞淋巴瘤继发噬血细胞综合征患者进行比较，综合分析其临床表现及相关实验室检查、疾病诊断所用时间、治疗方案及疾病转归等差异。结果6例患者均确诊噬血细胞综合征，均有发热、脾大、铁蛋白升高、噬血现象、肝功能异常，从确诊噬血细胞综合征到最终确诊淋巴瘤历时5~18个月。1例患者放弃治疗死亡，其余患者接受 CHOP 方案为主的化疗方案，1例患者序贯自体造血干细胞移植，2例患者接受异基因造血干细胞移植。除1例患者因 GVHD 死亡，其余患者随访期内病情稳定。结论皮下脂膜炎样 T 细胞淋巴瘤继发噬血细胞综合征临床少见，要积极控制噬血细胞综合征相关症状，以明确原发病诊断，此类患者疾病进展迅速，可考虑行大剂量化疗联合自体造血干细胞移植或异基因造血干细胞移植治疗。
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