【摘要】 目的研究儿童再生障碍性贫血及骨髓增生异常综合征患儿体内TCRδ基因克隆性重排及在免疫抑制治疗后的变化规律。方法收集2010年8月至2013年7月收治的59例儿童再生障碍性贫血及39例儿童骨髓增生异常综合征骨髓标本,使用巢式PCR检测TCRδ基因重排。结果 (1)再生障碍组59例共有6例(10.17%)TCRδ基因重排阳性,MDS-RCC组34例中6例(17.65%)阳性,5例MDS-JMML中无阳性。(2)再生障碍与骨髓增生异常综合征组TCRδ基因重排阳性率无统计学差异(P>0.05)。(3)再生障碍组中1例首次初筛阳性患者复发后TCRδ基因重新转阳,骨髓增生异常综合征组中2例初筛阳性患者使用免疫抑制剂效差伴骨髓TCRδ基因重排持续阳性。结论儿童再生障碍性贫血及骨髓增生异常综合征中有部分患者可出现TCRδ基因克隆性重排,监测克隆性重排可能应用于监测免疫抑制治疗效果。更多还原

【Abstract】 Objective To investigate the incidence of T-cell receptor δ gene rearrangement in children with aplastic anemia or myelodysplastic syndrome and the change rule after the immunosuppressive therapy applied. Methods From 2010 to 2013, 59 patients with aplastic anemia and 38 patients with myelodysplastic syndrome were enrolled. These patients were detected clonal rearrangement of T-cell receptor δ gene by nested PCR. Results(1) There were 6(6/59,17.65%)patients in aplastic anemia group, 6(6/34,17.56%) patients in myelodysplastic syndrome group and no patient in juvenile chronic myelomonocytic leukemia group who were detected the clonal rearrangement of T-cell receptor δ gene.(2) There was no statistical significance(P>0.05) of the positive rate between patients with aplastic anemia and those with myelodysplastic syndrome.(3) The detection of the rearrangement of TCRδ gene turned positive in a patient with aplastic anemia relapsed. Two patients suffered from myelodysplastic syndrome had the persistent positive results of the TCRδ gene with the poor response to the immunosuppressive therapy. Conclusions The rearrangement of T-cell receptor δ gene could be detected in some patients with aplastic anemia or myelodysplastic syndrome. The monitor of clonal T-cell receptor δ rearrangement could be applied in the surveying the effect of immunosuppressive therapy.更多还原