Transplacentally acquired maternal T lymphocytes in severe combined immunodeficiency:: a study of 121 patients
Transplacentally acquired maternal T lymphocytes in severe combined immunodeficiency:: a study of 121 patients
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DOI：
10.1182/blood.v98.6.1847
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发表时间：
2001-09-15
期刊：
影响因子：
20.3
通讯作者：
Friedrich, W
中科院分区：
文献类型：
--
作者：
Müller, SM;Ege, M;Friedrich, W
对 121 名患有严重联合免疫缺陷 (SCID) 的婴儿进行了一项研究，以确定经胎盘获得的母体 T 细胞植入的发生率，并探讨与这种异常相关的临床和免疫学发现。通过对 T 细胞和非 T 细胞进行选择性 HLA 分型，对每位新诊断的 SCID 循环 T 细胞患者进行嵌合状态评估。在植入的患者中，母体 T 细胞具有表型和功能特征，结果与患者的临床发现相关。在 48 名患者的循环中检测到母体 T 细胞；这些细胞的范围从 14 例低于 100/μL 到 4 例超过 2000/μL（中位数为 415/μL）。 29 名患者没有出现移植物抗宿主病 (GVHD) 的临床症状。其他病例出现 GVHD 表现，累及皮肤，其中 14 例还累及肝脏。 8 名患者的皮肤 GVHD 较轻。在这些患者以及没有 GVHD 迹象的患者中，母体 T 细胞主要为 CD8(+)，除一名例外，对丝裂原刺激没有反应。 9例患者皮肤GVHD表现突出。这些病例中的 T 细胞主要是 CD4(+)，并且对有丝分裂原刺激有反应，但有一个例外。在 8 例具有显着皮肤 GVHD 的病例中，潜在的 SCID 变异的特点是缺乏 B 细胞。在这项研究中，进一步了解了 SCID 临床和免疫学结果显着异质性的现象。
A study in 121 infants with severe combined immunodeficiency (SCID) was performed to determine the prevalence of an engraftment by transplacentally acquired maternal T cells and to explore clinical and immunological findings related to this abnormality. Each newly diagnosed patient with SCID presenting with circulating T cells was evaluated for chimerism by performing selective HLA typing of T cells and non-T cells. In patients with engraftment, maternal T cells were characterized phenotypically and functionally, and results were correlated with clinical findings in the patients. Maternal T cells were detected in the circulation in 48 patients; these cells ranged from fewer than 100/muL in 14 cases to more than 2000/muL in 4 cases (median, 415/muL). Clinical signs of graft-versus-host disease (GVHD) were absent in 29 patients. In the other cases, manifestations of GVHD were present, involving the skin and in 14 cases also the liver. Skin GVHD was mild in 8 patients. In these patients, as well as in patients with no signs of GVHD, maternal T cells were predominantly CD8(+) and, with one exception, failed to respond to mitogen stimulation. In 9 patients, manifestations of skin GVHD were prominent. T cells in these cases were predominantly CD4(+) and responded, with one exception, to mitogen stimulation. In 8 of the cases with prominent skin GVHD, the underlying SCID variant was characterized by the absence of B cells. In this study, further understanding is provided of a phenomenon that is responsible for the significant heterogeneity of clinical and immunological findings in SCID.