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Clonally expanded CD3+, CD4−, CD8− cells bearing the or the T-cell receptor in patients with the lymphoproliferative disease of granular lymphocytes

1991; Academic Press; Volume: 60; Issue: 3 Linguagem: Inglês

10.1016/0090-1229(91)90094-q

1090-2341

Franco Pandolfi, Robert Foa, Giulio Rossi, Renato Zambello, Teodoro Chisesi, Paola Francia di Celle, Nicola Migone, Giulia Casorati, Elisa Scarselli, Fabrizio Ensoli, Livio Trentin, Gianpietro Semenzato,

Lymphoma Diagnosis and Treatment

Among 60 retrospectively assessed patients with the lymphoproliferative disease of granular lymphocytes (LDGL), lymphocytes from only 2 patients had the CD3+, CD4−, CD8− phenotype, rarely observed in normal peripheral blood lymphocytes (about 3%). In this paper we report a detailed study of lymphocytes isolated from these two patients. The cells from patients 1 had the CD3+, CD4−, CD8−, WT31−, βF1−, TCRδ1+, TiγA−, BB3+, CD7+, CD16−, CD57+ phenotype, while cells from patient 2 had a phenotype even more rarely observed on normal lymphocytes: CD3+, CD4−, CD8−, WT31+, βF1+, TCRδ1−, CD7+, CD16−, CD57+. Thus, in only the first case the cells expressed the γδ T-cell receptor (TCR) on the membrane, while the cells from the second case had the αβ TCR. Genetic studies showed that in case 1 the TCR γ gene was rearranged and the β chain gene configuration was germline; the TCR mRNA was of normal size for the γ chain, while that of the β chain was truncated. Case 2 had the β and the γ genes of the TCR rearranged, but only the α and β mRNA were expressed. In agreement with these findings, the δ chain gene of the TCR was rearranged in case 1 and was deleted in case 2. Cytotoxic activity was absent in cells from case 1 and low in case 2; in the latter, the lytic activity could be up-regulated following incubation with IL-2 or an anti-CD3 monoclonal antibody. Our study indicates that CD3+, CD4−, CD8− lymphocytes are rarely expanded in patients with LDGL. The detection of a lymphoproliferative disease of a CD3+, CD4−, CD8−, αβ+ cell may contribute to a better characterization of this novel lymphocytic subpopulation.