Highly restricted human T cell repertoire in peripheral blood and tissue-infiltrating lymphocytes in Omenn's syndrome

被引：67

作者：

Rieux-Laucat, F

Bahadoran, P

Brousse, N

Selz, F

Fischer, A

Le Deist, F

De Villartay, JP

机构：

[1] Hop Necker Enfants Malad, INSERM, U429, F-75743 Paris 15, France

[2] Hop Necker Enfants Malad, Serv Anatomopathol, F-75743 Paris 15, France

来源：

JOURNAL OF CLINICAL INVESTIGATION | 1998年 / 102卷 / 02期

关键词：

combined immunodeficiency; Omenn syndrome; T lymphocytes; HTCRBV; repertoire;

D O I：

10.1172/JCI332

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

Omenn's syndrome is an inherited human combined immunodeficiency condition characterized by the presence of a large population of activated and tissue-infiltrating T cells. Analysis of the TCRB repertoire revealed a highly restricted TCRBV usage in three patients. More strikingly, T cell clones from the three patients expressed TCRB chains with VDJ junction similarities, suggesting a common antigenic specificity. Analysis of the TCRA repertoire in one patient also revealed a restricted TCRAV usage. Finally, analysis of the TCRBV repertoire of tissue-infiltrating T cells in one patient suggested nonrandom tissue migration, These results suggest that the oligoclonal expansion of T cells observed in Omenn's syndrome could be the consequence of autoimmune proliferation generated by a profound defect in lymphocyte development.

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收藏

页码：312 / 321

页数：10

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