大剂量化疗结合外周血自体干细胞移植治疗系统性红斑狼疮

Therapeutic effect of high-dose chemotherapy combined with autologous peripheral blood stem cell transplantation on systemic lupus erythematosus

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 explore the clinical effect by high dose chemotherapy with autologous peripheral blood stem cell transplantation (APBSCT) on refractory systemic lupus erythematosus (RSLE).Methods Six patients with RSLE were chosen.The patients were firstly given plasma exchange,cyclophosphamide (CTX) and high dosage IgG;peripheral blood stem cell (PBSCs) were mobilized with CTX and granulocyte colony stimulating factor (G CSF).The haematopoietic stem cells (HSCs) harvesting was performed by continuous flow leukapheresis using a MCS plus blood separator.The cells were preserved at ultra low temperature(-86 ℃).PBSCs were reinfused after conditioned by CTX (50 mg/kg),granulocyte recovery was stimulated by G CSF,antithymocyte globulin (ATG) was given in vivo for T cell depletion.Both clinical manifestations and immunologic markers were compared before and after transplantation.Results After transplantation the patients received clinical remission and their immunologic abnormality returned to negative.Conclusion APBSCT is effective and safety for RSLE.It is required to follow up for evaluating long term effect.