Long-term T-cell reconstitution after hematopoietic stem-cell transplantation in primary T-cell-immunodeficient patients is associated with myeloid chimerism and possibly the primary disease phenotype
Long-term T-cell reconstitution after hematopoietic stem-cell transplantation in primary T-cell-immunodeficient patients is associated with myeloid chimerism and possibly the primary disease phenotype
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DOI：
10.1182/blood-2006-07-029090
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发表时间：
2007-05-15
期刊：
影响因子：
20.3
通讯作者：
Hacein-Bey-Abina, Salima
中科院分区：
文献类型：
--
作者：
Cavazzana-Calvo, Marina;Carlier, Frederique;Hacein-Bey-Abina, Salima
我们研究了 31 名原发性 T 细胞免疫缺陷患者的 T 细胞重建，这些患者在 10 多年前接受过造血干细胞移植 (HSCT)。在 19 名患者中，没有骨髓嵌合的证据，因为很少或没有进行清髓术。鉴于这种背景，我们寻找与良好的长期 T 细胞重建相关的因素。我们发现，所有接受全清髓术的患者都具有供体骨髓细胞和持续的胸腺生成功能，携带 T 细胞受体切除环 (TREC) 的初始 T 细胞的存在就证明了这一点。在 9 名具有宿主骨髓嵌合状态的患者中，还观察到持续的胸腺输出，并且似乎与 γc 缺乏有关。因此，这种情况下胸腺祖细胞的完全缺失可能为具有自我更新潜力的早期祖细胞群体的胸腺播种创造了更有利的环境，从而实现了长期（> 10年）T细胞的产生。
We studied T-cell reconstitution in 31 primary T-cell-immunodeficient patients who had undergone hematopoietic stem-cell transplantation (HSCT) over 10 years previously. In 19 patients, there was no evidence of myeloid chimerism because little or no myeloablation had been performed. Given this context, we sought factors associated with good long-term T-cell reconstitution. We found that all patients having undergone full myeloablation had donor myeloid cells and persistent thymopoiesis, as evidenced by the presence of naive T cells carrying T-cell receptor excision circles (TRECs). In 9 patients with host myeloid chimerism, sustained thymic output was also observed and appeared to be associated with gamma c deficiency. It is therefore possible that the complete absence of thymic progenitors characterizing this condition created a more favorable environment for thymic seeding by a population of early progenitor cells with the potential for self-renewal, thus enabling long-term (> 10 years) T-cell production.