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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >T-cell receptor Vbeta repertoire analysis in patients with chronic idiopathic neutropenia demonstrates the presence of aberrant T-cell expansions.
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T-cell receptor Vbeta repertoire analysis in patients with chronic idiopathic neutropenia demonstrates the presence of aberrant T-cell expansions.

机译：慢性特发性中性粒细胞减少症患者的T细胞受体Vbeta库分析表明存在异常T细胞扩增。

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摘要

Chronic idiopathic neutropenia (CIN) is a bone marrow (BM) disorder characterized by presence of activated T-lymphocytes in peripheral blood (PB) and BM. We investigated the pattern of T-cell responses in CIN by analyzing the T-cell receptor beta-chain variable (Vbeta) gene repertoire. Compared to controls, CIN patients displayed different patterns of Vbeta gene usage in PB CD3(+), CD4(+) and CD8(+) cells. The frequency of Vbeta skewing and the number of expanded Vbeta families per subject were higher in patients compared to controls in all cell subpopulations. Skewing was more profound within the CD8(+) cells. The number of Vbeta expansions per patient was higher in BM compared to PB. The majority of patients displayed a skewed oligoclonal/monoclonal pattern within the PB and/or BM CD8(+) cells and a polyclonal profile within the CD4(+) cells. We concluded that aberrant T-cell expansions are invariably detected in CIN patients and may have a role in the disease pathogenesis.
机译：慢性特发性中性粒细胞减少症（CIN）是一种骨髓（BM）疾病，其特征在于外周血（PB）和BM中存在活化的T淋巴细胞。我们通过分析T细胞受体β链可变（Vbeta）基因库来研究CIN中T细胞反应的模式。与对照组相比，CIN患者在PB CD3（+），CD4（+）和CD8（+）细胞中显示出不同的Vbeta基因使用模式。与所有细胞亚群中的对照相比，患者的Vbeta偏斜频率和每个受试者扩展的Vbeta家族数目更高。倾斜在CD8（+）细胞中更为深刻。与PB相比，BM的每位患者的Vbeta扩展数更高。大多数患者在PB和/或BM CD8（+）细胞内显示偏斜的寡克隆/单克隆模式，在CD4（+）细胞内显示多克隆谱。我们得出的结论是，在CIN患者中始终检测到异常T细胞扩增，并且可能在疾病发病机理中起作用。

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