儿童白血病造血干细胞移植后免疫性血细胞减少症3例分析

目的探讨白血病造血干细胞移植后免疫性血细胞减少症发生的影响因素,治疗和预后。方法对3例造血干细胞移植后长期存活并发生免疫性血细胞减少症的白血病患儿的临床资料进行回顾性分析。结果在19例移植后长期存活的患儿中,3例发生免疫性血细胞减少症,发生率为16%,男2例,女1例,分别发生在移植后+180 d、+186 d和+384 d;发生年龄分别为8岁、9岁8个月和9岁11个月;1例HLA 6/6相合,2例为5/6相合;均采用无关脐血移植,在预处理中使用兔抗人胸腺细胞免疫球蛋白(ATG)。仅有1例发生移植物抗宿主病(GVHD),2例在植入早期发生巨细胞病毒感染。3例均接受以糖皮质激素为基础的免疫抑制治疗,2例治疗反应良好,停药后无复发,1例需免疫抑制剂维持。结论在本研究中,性别、HLA配型差异、供受者血型不合、GVHD不是造血干细胞移植后免疫性血细胞减少症的危险因素。脐血移植、使用ATG和早期病毒感染可能与造血干细胞移植后免疫性血细胞减少症的发生有关,本病主要治疗手段为以糖皮质激素为基础的免疫抑制治疗。

Analysis of autoimmune cytopenias after hematopoietic stem cell transplantation in childhood leukemia

Objective To analyze the incidence and risk factors as well as its prognosis and treatment in the development of autoimmune cytopenias after hematopoietic stem cell transplantation(HSCT) in childhood leukemia.Methods Nineteen children with leukemia after HSCT were retrospectively analyzed.Results Three children developed autoimmune cytopenias at +180,+186 and +384 day after HSCT.The incidence of autoimmune cytopenias was 15.78%,including 2 males and 1 female at the age of 8,9 years 8 months,and 9 years 11 months.Among them,one had a HLA match(6/6) and others had a HLA mismatch(5/6);only one patient developed GVHD,and the other two developed CMV infections after HSCT;all patients underwent unrelated cord blood transplantation with pretreatment of ATG.All patients received immunosuppressive therapy based on prednisone.Two of them had a good therapeutic reaction and stopped immunosuppressive therapy,and one needed maintaining the immunosuppressive therapy.Conclusions Gender,HLA disparity,ABO mismatch and GVHD are not the risk factors for the development of autoimmune cytopenias after HSCT.Unrelated cord blood transplantation and pretreatment with ATG may be the potential risk factors of the autoimmune cytopenias after HSCT,and need more investigations.Immunosuppressive therapy based on the steroids is the major treatment.