免疫清除性化疗结合自体外周血造血干细胞移植治疗重症系统性红斑狼疮

Immunoablative chemotherapy with autologous peripheral blood stem cell rescule in patients with severe systemic lupus erythematosus.

目的 观察免疫清除性化疗结合自体外周血造血干细胞移植 (移植 )治疗重症系统性红斑狼疮 (SLE)的疗效及安全性。方法 重症SLE 4例 ,分别合并狼疮性肾炎、狼疮脑、狼疮心或股骨头坏死 ,干细胞的动员采用环磷酰胺加重组人粒细胞集落因子 (G CSF) ;预处理为回输前 3天每天应用环磷酰胺 5 0mg/kg +回输后 3天每天应用抗胸腺细胞球蛋白 (ATG) 5mg/kg。观察移植前后临床症状、体征、狼疮相关抗体等指标的改变 ,并动态观察移植后免疫功能的重建。结果 移植后患者的临床症状完全缓解 ,狼疮相关抗体全部转阴 ,移植后患者的免疫功能均明显降低 ,约半年后恢复正常 ,但不伴有临床症状的复发。结论 免疫清除性化疗结合自体外周血造血干细胞移对难治性SLE有明显的疗效 ,尤其适用对各种药物治疗无效者 ,治疗是安全的 。

Objective To study the clinical effect of immunoablative chemotherapy with autologous peripheral blood stem cell rescule in patients with severe systemic lupus erythematosus (SLE).Methods Four severe SLE were chosen. Peripheral blood stem cell (PBSCs) were mobilized with CTX and granulocyte colony-stimulating factor (G CSF). PBSCs were reinfused after conditioned by CTX (50mg/kg every day for 3 days) + Antithymocyte globulin (5mg/kg every day for 3 days). Both clinical manifestations and lupus -correlated antigens were compared before and after transplantation. The reconstrution of immune function were observed.Results After transplantation the patients received clinical remission and immunologic abnormality returned to normal. Immune function decreased remarkably after the transplantation and resumed to normal about 6 months after the transplantation.Conclusion APBSCT is effective and safety for severe SLE. It is required to follow-up for evaluation long-term effect.. \[