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目的:探讨自体外周血造血干细胞移植(APBSCT)治疗难治性系统性红斑狼疮(SLE)的临床疗效。方法:用APBSCT治疗4例难治性SLE。干细胞动员应用环磷酰胺(CTX)4g/m2,分两天应用和粒细胞集落刺激因子(G-CSF)5~10μg/kg·d-1;预处理方案包括CTX(50mg/kg·d-1,-6、-5、-4、-3d),抗胸腺细胞球蛋白(ATG15~20mg/kg·d-1,-2、-1、+1、+2d)。结果:4例患者均获得造血重建,中性粒细胞>0.5×109/L,血小板>20×109/L的中位数时间分别是9d,10d;SLE的临床表现明显减轻,尿蛋白减少或消失,自身抗体转阴或滴度减低,泼尼松用量<10mg/d;无移植相关死亡。结论:APBSCT治疗难治性SLE近期疗效显著,造血重建恢复迅速,安全有效,远期疗效尚需进一步观察。
Objective: To investigate the clinical efficacy of autologous peripheral blood stem cell transplantation (APBSCT) in the treatment of refractory systemic lupus erythematosus (SLE). Methods: Four cases of refractory SLE were treated with APBSCT. Stem cell mobilization was performed using cyclophosphamide (CTX) at a dose of 4 g / m 2 for 5 days and 5 to 10 μg / kg · d -1 of granulocyte colony-stimulating factor (G-CSF) 1, -6, -5, -4, -3d), anti-thymocyte globulin (ATG15 ~ 20mg / kg · d-1, -2, -1, +1, +2 d). Results: The median time of hematopoietic reconstitution, neutrophil> 0.5 × 109 / L and platelet count> 20 × 109 / L was 9d and 10d respectively in all 4 patients. The clinical manifestations of SLE were significantly reduced, Disappeared, autoantibodies or negative titer decreased, prednisone dosage <10mg / d; no transplant-related deaths. Conclusion: APBSCT treatment of refractory SLE significant effect of short-term, rapid recovery of hematopoietic reconstructive, safe and effective long-term efficacy needs further observation.