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目的探讨治疗儿童难治型系统性红斑狼疮 (SLE)积极、有效的方法。方法对 2例病程分别为 5年和 7年 ,狼疮肾Ⅲ级和Ⅳ级 ,主要表现为持续性血小板减少、蛋白尿和浆膜炎的患儿进行CD34+ 细胞分选的自身干细胞移植。首先经惠尔血动员、CS 30 0 0血细胞分离机获取单个核细胞 ,通过CliniMACSCD34+ 细胞分选仪分别得到了 1.7× 10 6/kg及 1.0× 10 6/kgCD34+细胞 ,采集物中分别尚存 2× 10 5/kg和 1× 10 4/kg的CD3+ 细胞。用CTX 5 0mg/kg·d× 4d +ATG(FresenniusS 5mg/kg·d× 3d)预处理。结果两患者分别于 +9d和 +7d获粒细胞重建 ,自 +15d起血小板维持于正常水平。现已分别随访 13月和 6月 ,原发病症状完全消失 ,自身免疫相关抗体全部转阴 ,但细胞免疫功能仍未恢复 ,CD4细胞仍处于低水平。结论CD34+ 细胞分选的自身干细胞移植治疗儿童难治型红斑狼疮近期疗效满意。
Objective To explore a positive and effective method for the treatment of refractory systemic lupus erythematosus (SLE) in children. Methods Two patients with 5 and 7 years of disease course and lupus nephritis grade Ⅲ and Ⅳ, respectively, were mainly treated with self-stem cell transplantation of CD34 + cells in children with persistent thrombocytopenia, proteinuria and serositis. Monocytes were harvested from the hemocytes of CS30 × 0 cells after being mobilized by Wurr blood, and 1.7 × 10 6 / kg and 1.0 × 10 6 / kg of CD34 + cells were collected by CliniMACSCD34 + cell sorter respectively. × 10 5 / kg and 1 × 10 4 / kg of CD3 + cells. The cells were pre-treated with CTX 50 mg / kg.d4h + ATG (Fresennius S 5 mg / kg.d3d). Results The two patients got granulocyte reconstitution at + 9d and + 7d, respectively. Platelets were maintained at normal levels from + 15d. Now follow-up of 13 months and 6 months, the symptoms of the primary disease disappeared completely, all antibodies related to autoimmunity turned negative, but the cellular immune function was still not recovered and CD4 cells were still at a low level. Conclusion CD34 + cell sorting stem cell transplantation in children with refractory lupus erythematosus short-term curative effect is satisfactory.