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目的 研究、评价大剂量化疗结合自体外周血干细胞移植 (APBSCT)治疗难治性系统性红斑狼疮 (RSLE)的安全性和疗效。方法 6例活动性难治性系统性红斑狼疮病人 [男 2例 ,平均年龄 ( 2 3±4)岁 ,女 4例 ,平均年龄 ( 2 4± 7)岁 ]。 ( 1)先进行血浆置换 ,再用同步化免疫抑制剂 ;( 2 )输入环磷酰胺(CTX)后 ,待WBC下降到 1.0× 10 9 L时给予粒细胞集落刺激因子 (G CSF)动员造血干细胞 (HSC) ;通过MCS +型血细胞分离机采集外周血干细胞 ;( 3 )给予环磷酰胺预处理 ;( 4 )回输干细胞 ;( 5 )应用抗胸腺细胞球蛋白 (ATG)行体内去除T淋巴细胞。检测观察病人的副反应及造血和免疫功能的重建。结果 6例病人都成功地进行了外周血干细胞移植的全过程 ,造血功能及免疫功能迅速恢复 ,未观察到严重的毒性反应及并发症。亦对移植病人进行了密切随访已 3～ 18个月 ,现已分别恢复家务、工作和学习。结论 APBSCT对难治性系统性红斑狼疮有明显的疗效 ,尤其适用对各种药物治疗无效的患者 ,治疗是安全的 ,远期疗效还需长期随访。
Objective To evaluate the safety and efficacy of high-dose chemotherapy combined with autologous peripheral blood stem cell transplantation (APBSCT) in the treatment of refractory systemic lupus erythematosus (RSLE). Methods Six patients with active refractory systemic lupus erythematosus (2 males, mean age (23 ± 4) years and 4 females, mean age (24 ± 7) years) were included in this study. (1) first plasma exchange and then synchronized immunosuppressive agents; (2) after the introduction of cyclophosphamide (CTX), mobilize granulocyte colony-stimulating factor (G CSF) to mobilize hematopoietic when WBC decreased to 1.0 × 10 9 L Stem cells (HSC); peripheral blood stem cells were collected by MCS + type blood cell separator; (3) pretreatment with cyclophosphamide; (4) transfusion of stem cells; (5) T cells were removed in vivo using antithymocyte globulin Lymphocytes. Detection and observation of patients with side effects and reconstruction of hematopoietic and immune function. Results All the 6 patients successfully performed the whole process of peripheral blood stem cell transplantation. The hematopoietic function and immune function recovered rapidly. No serious toxic reactions and complications were observed. A close follow-up of the transplant patients has also been conducted for 3 to 18 months, and housekeeping, work and study have been resumed respectively. Conclusion APBSCT has obvious curative effect on refractory systemic lupus erythematosus. It is especially suitable for patients who are ineffective in all kinds of medical treatment. The treatment is safe and long-term follow-up is needed.