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A Sottini, A Bettinardi, E Quiros-Roldan, A Plebani, P Airo, D Primi and L Imberti
Terzo Laboratorio Analisi and Clinical Immunology, Consorzio per le
Biotecnologie, Spedali Civili, Brescia, Italy.
It is now recognized that CD3+ large granular lymphocyte (LGL)
proliferations may be clonally derived from their normal CD3+LGL+
counterpart, but the nature of the pressure responsible for the
proliferation of these cells remains unclear. We approached this problem by
analyzing the diversity of the T-cell receptor repertoire of LGL developed
in different clinical settings. Two of our patients had typical
lymphoproliferative disorders. The third case was much more unusual, as the
LGL proliferation was associated with a Wiskott-Aldrich syndrome. Our data
relative to the patients with the lymphoproliferative disorders only
suggest that these LGL were clonally expanded. The data relative to the
patient with Wiskott-Aldrich syndrome were more unexpected, as the T-cell
repertoire of the LGL appeared to have common features with that of the
other T-cell populations analyzed. These LGL were characterized by the
clonal expansion of a few TCRBV segments that shared common amino acid
motifs in the junctional region of the T-cell receptor. This common pattern
of junctional diversity associated with different TCRBV segments is,
therefore, consistent with a strong on-going antigenic selection process,
possibly related to the pathogenesis of Wiskott-Aldrich syndrome.
Furthermore, the finding that the same TCRBV segments were also highly
expanded among other T-cell subpopulations questions the malignant nature
of this LGL proliferation.
This article has been cited by other articles:
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| Copyright © 1995 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
