Published in Volume
102, Issue 2 (July 15,1998)
J. Clin. Invest.
102(2):
312-321 (1998).
doi:10.1172/JCI332.
Copyright ©
1998, The American Society for
Clinical Investigation.
Research Article
Highly restricted human T cell repertoire in peripheral blood and tissue-infiltrating lymphocytes in Omenn's syndrome.
F Rieux-Laucat,
P Bahadoran,
N Brousse,
F Selz,
A Fischer,
F Le Deist and
J P De Villartay
Unité INSERM 429, Hôpital Necker-Enfants Malades, 75743 Paris, Cedex 15, France. rieux@necker.fr
Published July 15,
1998
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.
