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目的探讨自体外周血纯化造血干细胞移植治疗系统性红斑狼疮(SLE)的疗效和安全性。方法对9例SLE患者进行自体外周血纯化造血干细胞移植。采集的干细胞的计数为(2．37～9．9)×10~8/ kg。预处理方案是环磷酰胺50 mg·kg~(-1)·d~(-1)静脉滴注,连用4 d(造血干细胞回输前2～5d)。抗胸腺球蛋白抗体2．5 mg·kg~(-1)·d~(-1)静脉滴注,连用4 d。同时碱化和水化尿液,保护心、肝和肾功能。从移植后临床表现、SLE相关的免疫学指标的变化,移植后造血重建情况,移植的并发症等方面进行评价。结果9例患者均获得成功植入,外周血白细胞总数＞1．0×10~9/L的时间为移植后7～15 d,血小板＞20×10~9/L时间为移植后0～21d。所有患者均于移植后面部红斑等临床症状完全消失,大部分患者自身抗体转阴。9例患者均出现轻重不一的血清病样反应,1例出现严重的肾衰和心衰,3例有出血性膀胱炎,1例出现心因性精神障碍,1例发生会阴部念珠菌感染。结论随访1年结果表明,自体外周血纯化造血干细胞移植治疗SLE的近期疗效显著,且相对安全。
Objective To investigate the efficacy and safety of autologous peripheral blood stem cell transplantation in the treatment of systemic lupus erythematosus (SLE). Methods Nine patients with SLE underwent autologous peripheral blood hematopoietic stem cell transplantation. The count of collected stem cells was (2.37 ~ 9.9) × 10 ~ 8 / kg. Pretreatment regimen was cyclophosphamide (50 mg · kg -1 d -1) intravenously for 4 d (2 ~ 5 d before hematopoietic stem cell reinfusion). Anti-thymoglobulin antibody 2.5 mg · kg -1 d -1 intravenous infusion for 4 d. At the same time alkaline and hydration of urine, protect the heart, liver and kidney function. From the clinical manifestations after transplantation, SLE-related immunological changes, hematopoietic reconstitution after transplantation, complications of transplantation and other aspects of evaluation. Results All the 9 patients were successfully implanted. The total number of peripheral blood leukocytes> 1.0 × 10 ~ 9 / L was 7-15 days after transplantation, and the platelet count> 20 × 10-9 / L was 0-21 days after transplantation . All patients had complete disappearance of clinical symptoms such as erythema on the face after transplantation. Most of the patients had negative autoantibodies. Nine patients had varying severity of serum-like reactions, one with severe renal failure and heart failure, three with hemorrhagic cystitis, one with psychogenic disorders, and one with peritoneal candidiasis . Conclusions One year follow-up results show that the short-term curative effect of SLE with autologous peripheral blood purification is significant and relatively safe.