克隆性TCR基因重排在获得性纯红细胞再生障碍性贫血的意义  

Significance of clonai TCR gene rearrangement in acquired pure red cell aplastic anemia

作　　者：王增胜[1] 安利[1] 木舍塔拜尔 张晓燕[1] 富玲[1] 王晓敏[1] 

机构地区：[1]新疆维吾尔自治区人民医院血液科,乌鲁木齐830020

出　　处：《中华医学遗传学杂志》2016年第3期369-372,共4页Chinese Journal of Medical Genetics

摘　　要：目的探讨获得性纯红细胞再生障碍性贫血（acquired pure red cell aplastic anemia，A-PRCA）患者中T细胞抗原受体（T cell antigen receptor，TCR）基因克隆性重排的意义。方法对16例A—PRCA患者，应用以环胞素菌A为基础的免疫抑制方案治疗，用PCR检测TCR基因重排，用流式细胞仪对外周血标本进行T淋巴细胞亚群检测。结果16例患者中，有5例检测到砌℃克隆性重排，且5例均为TCR7和TCR8阳性。16例患者中，13例血象恢复正常（3例骨髓象同时恢复正常），7例骨髓象红系增生仍减低，但较治疗前有所上升。基本治愈3例，缓解7例，改善3例，无效3例，总有效率为81．25％。其中3例无效者均为TCR重排阳性患者，TCR重排阴性患者组治疗有效比例（11／11）明显高于TCR克隆性重排阳性患者组（2／5），差异有统计学意义（χ^2=8．123，P=0．018）。TCR重排阳性患者组的Th细胞（CD3＋CD4＋），Th／Ts比值均低于TCR克隆性重排阴性患者组，差异有统计学意义（P〈O．05）；而Ts细胞（CD3＋CD8＋）细胞的表达高于TCR克隆性重排阴性患者组，差异有统计学意义（P〈0．05）。结论部分A-PRCA患者存在TCR克隆性重排，其在A-PRCA发病机制中可能起一定的作用。免疫抑制剂环胞菌素A对A—PRCA有较好的疗效，但存在TCR克隆性重排者对环胞素菌A治疗反应差。Objective To evaluate the significance of T-cell antigen receptor （TCR） gene rearrangement among patients with acquired pure red cell aplastic anemia （A-PRCA）. Methods For 16 patients with A-PRCA, an immunosuppressive regimen based on cyclosporin A （CsA） was applied. Rearrangement of the TCR gene was detected by PCR, and T lymphocyte subsets in peripheral blood specimens was detected with flow cytometry. Results Five patients had presented with TCR clonal rearrangement and were positive for both TCRγ and TCRδ The blood of 13 patients have returned to normal with the treatment, which included 3 cases with bone marrow returning to normal. In 7 cases, the red cell hyperplasia of hone marrow is still down, but has increased with the treatment. Three patients were close to cure, 7 showed remission, 3 were improved, but 3 were ineffective. The rate of effective treatment in those with TCR rearrangement （2/5） was significantly lower than that those without （11/11, χ^2=8. 123, P〈0.05）. Compared with those without the TCR gene rearrangement, the Th cells and proportion of Th/Ts were significantly lower, while the Ts cell （CD3 ＋CD8＋ ） were significantly higher in those with the rearrangement （P〈0.05）. Conclusion TCR gene rearrangement may play a role in the pathogenesis of A-PRCA. CsA is effective for the treatment of A-PRCA, but patients presenting clonal TCR gene rearrangement may response poorly to the treatment.

关 键 词：获得性纯红细胞再生障碍性贫血 TCR基因重排 T淋巴细胞亚群 

分 类 号：R556.5[医药卫生—血液循环系统疾病]