Autoimmune hemolytic anemia in patients with SCID after T cell-depleted BM and PBSC transplantation
Autoimmune hemolytic anemia in patients with SCID after T cell-depleted BM and PBSC transplantation
复制标题
DOI：
10.1038/sj.bmt.1702011
复制
发表时间：
1999-11-01
影响因子：
4.8
通讯作者：
Cowan, M
中科院分区：
文献类型：
--
作者：
Horn, B;Viele, M;Cowan, M
我们报告了41例接受T细胞耗尽的单倍体相合移植的SCID患者中，自身免疫性溶血性贫血(AIHA)的发生率很高(19.5%)，除了感染，AIHA是该患者队列中最常见的移植后并发症。报告了8例首次T细胞耗竭移植后平均8个月出现AIHA的患者的临床特征和治疗方法。所有患者都有热反应自身抗体，8名患者中有2名同时患有冷热自身抗体。2例冷热自身抗体阳性患者的临床病程最为严重。5名患者接受了针对AIHA的特殊治疗。成功地停止免疫抑制治疗AIHA与T细胞重建是一致的。T细胞耗竭、免疫抑制调节和CsA导致的T细胞免疫重建延迟，以及调节性T细胞的缺乏，可能是我们的患者队列中发生AIHA的可能原因。对高危人群进行筛查可以预防AIHA的发病率和死亡率。
We report a high incidence (19.5%) of autoimmune hemolytic anemia (AIHA) in 41 patients with SCID who underwent a T cell-depleted haploidentical transplant, Other than infections, AIHA was the most common posttransplant complication in this patient cohort. Clinical characteristics and treatment of eight patients who developed AIHA at a median of 8 months after the first T cell-depleted transplant are presented. All patients had warm-reacting autoantibodies, and two of eight had concurrent cold and warm autoantibodies. Clinical course was most severe in two patients who had cold and warm autoantibodies. Five patients received specific therapy for AIHA. Successful taper off immunosuppressive therapy for AIHA coincided with T cell reconstitution. Delayed reconstitution of T cell immunity, due to T cell depletion, immunosuppressive conditioning and CsA, as well as paucity of regulatory T cells, are the likely explanations for the occurrence of AIHA in our patient cohort. Screening of the population at risk may prevent morbidity and mortality from AIHA.