中文摘要：

目的探讨难治性天疱疮患者自体外周血干细胞移植（APBSCT）前、后T细胞抗原受体B链（TCRl3）互补决定区3（CDR3）基因表达谱型变化，深入了解天疱疮发病机制以及患者造血干细胞移植后T细胞克隆的免疫重建特点。方法应用RT—PCR方法扩增天疱疮患者移植前、后外周血单个核细胞24个TCRl3CDR3区段基因序列，分析其基因表达情况。采用免疫扫描谱型分析技术，分析正常人外周血单核细胞TCRl3链CDR3谱型分布特征，以及难治性天疱疮患者干细胞移植前、后CDR3谱型变化情况。结果动员前天疱疮患者TCRl3CDR3谱型均出现异常，表现为一定程度的T细胞单（寡）克隆增生性表达；移植后12个月内仍出现部分表达缺失及寡克隆表达；而后T细胞克隆分布渐趋于正常，CDR3恢复多态性。结论难治性天疱疮患者外周血T细胞表现单（寡）克隆增生，CDR3多态性降低，可能与其发病机制有关。APBSCT后的免疫功能重建恢复了CDR3的多态性，提示造血干细胞移植是治疗难治性天疱疮的有效手段。

英文摘要：

Objective To explore the variation of the complementarity-determining region 3 （CDR3） of T cell receptor（TCR） β chain variable region（ TCR BV） in peripheral blood mononaclear cells（PBMC） of patients with refractory pemphigus before and after autologous peripheral blood stem cell transplantation（ APBSCT）, so as to elucidate the pathogenesis of pemphigus and characteristics of immunoreconstitution of peripheral T lymphocytes after APBSCT. Methods Before and after APBSCT,24 subfamily genes of TCR β chain from peripheral blood lymphocyte of pemphigus patients with APBSCT were amplified by reverse transcriptase-polymerase chain reaction（RT-PCR） and the spectratype distribution of TCR BV genes was compared between normal blood donors and pemphigus patients, using immunoscope spectratyping technique. Results Before APBSCT, the CDR3 spectratypes of TCR BV subfamily in pemphigus patients showed abnormality with monoclonal or oligoclonal proliferation of T cells, which could maintain within 12 months after APBSCT,and then T cell clonal diversity recovered gradually, especially post-PBSCT follow-up for 36 months later. Conclusion T lymphocytes in pemphigus appear monoclonal or oligoclonal proliferation with a reduction of CDR3 diversity, which might be involved in the pathogenesis of pemphigus. Recovery of CDR3 diversity of T cell due to immunological reconsititution following APBSCT suggests that APBSCT would be an effective way to the treatment of pemphigus.