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首页> 外文期刊>Journal of immunology research. >Long-Lasting Production of New T and B Cells and T-Cell Repertoire Diversity in Patients with Primary Immunodeficiency Who Had Undergone Stem Cell Transplantation: A Single-Centre Experience
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Long-Lasting Production of New T and B Cells and T-Cell Repertoire Diversity in Patients with Primary Immunodeficiency Who Had Undergone Stem Cell Transplantation: A Single-Centre Experience

机译：曾接受干细胞移植的原发性免疫缺陷患者的新T和B细胞的长期生产和T细胞库多样性：单中心经验

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Levels of Kappa-deleting recombination excision circles (KRECs), T-cell receptor excision circles (TRECs), and T-cell repertoire diversity were evaluated in 1038 samples of 124 children with primary immunodeficiency, of whom 102 (54 with severe combined immunodeficiency and 48 with other types of immunodeficiency) underwent hematopoietic stem cell transplantation. Twenty-two not transplanted patients with primary immunodeficiency were used as controls. Only data of patients from whom at least five samples were sent to the clinical laboratory for routine monitoring of lymphocyte reconstitutions were included in the analysis. The mean time of the follow-up was 8 years. The long-lasting posttransplantation kinetics of KREC and TREC production occurred similarly in patients with severe combined immunodeficiency and with other types of immunodeficiency and, in both groups, the T-cell reconstitution was more efficient than in nontransplanted children. Although thymic output decreased in older transplanted patients, the degree of T-cell repertoire diversity, after an initial increase, remained stable during the observation period. However, the presence of graft-versus-host disease and ablative conditioning seemed to play a role in the time-related shaping of T-cell repertoire. Overall, our data suggest that long-term B- and T-cell reconstitution was equally achieved in children with severe combined immunodeficiency and with other types of primary immunodeficiency.
机译：在124例原发性免疫缺陷儿童的1038份样本中评估了删除Kappa重组切除环（KRECs），T细胞受体切除环（TRECs）和T细胞库多样性的水平，其中102例（54例合并严重免疫缺陷和48例具有其他类型免疫缺陷的患者）进行了造血干细胞移植。将22例原发性免疫缺陷的未移植患者用作对照。分析中仅包括至少五个样本被送往临床实验室常规监测淋巴细胞重构的患者数据。随访的平均时间为8年。患有严重合并免疫缺陷和其他类型免疫缺陷的患者，KREC和TREC产生的持久移植后动力学类似地发生，并且在两组中，T细胞重建的效率均高于未移植的儿童。尽管在较老的移植患者中胸腺输出减少，但是在最初观察到之后，T细胞库多样性的程度在最初增加后仍保持稳定。然而，移植物抗宿主病和消融条件的存在似乎在与时间相关的T细胞库形成中起作用。总体而言，我们的数据表明，患有严重的合并免疫缺陷和其他类型的原发性免疫缺陷的儿童可以长期实现B细胞和T细胞的重建。

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