Pandolfi F, Foa R, De Rossi G, Zambello R, Chisesi T, di Celle P F, Migone N, Casorati G, Scarselli E, Ensoli F
Department of Allergy and Clinical Immunology, La Sapienza University of Rome, Italy.
Clin Immunol Immunopathol. 1991 Sep;60(3):371-83. doi: 10.1016/0090-1229(91)90094-q.
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-, beta F1-, TCR delta 1+, Ti gamma A-, BB3+, CD7+, CD16-, CD57+ phenotype, while cells from patient 2 had a phenotype even more rarely observed on normal lymphocytes: CD3+, CD4-, CD8-, WT31+, beta F1+, TCR delta 1-, CD7+, CD16-, CD57+. Thus, in only the first case the cells expressed the gamma/delta T-cell receptor (TCR) on the membrane, while the cells from the second case had the alpha/beta TCR. Genetic studies showed that in case 1 the TCR gamma gene was rearranged and the beta chain gene configuration was germline; the TCR mRNA was of normal size for the gamma chain, while that of the beta chain was truncated. Case 2 had the beta and the gamma genes of the TCR rearranged, but only the alpha and beta mRNA were expressed. In agreement with these findings, the delta 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-, alpha/beta + cell may contribute to a better characterization of this novel lymphocytic subpopulation.
在60例经回顾性评估的颗粒淋巴细胞淋巴增殖性疾病（LDGL）患者中，仅2例患者的淋巴细胞具有CD3 +、CD4 -、CD8 -表型，这种表型在正常外周血淋巴细胞中很少见（约3%）。在本文中，我们报告了对从这两名患者分离出的淋巴细胞的详细研究。患者1的细胞具有CD3 +、CD4 -、CD8 -、WT31 -、βF1 -、TCRδ1 +、TiγA -、BB3 +、CD7 +、CD16 -、CD57 +表型,而患者2的细胞具有一种在正常淋巴细胞上更罕见的表型：CD3 +、CD4 -、CD8 -、WT31 +、βF1 +、TCRδ1 -、CD7 +、CD16 -、CD57 +。因此，仅在第一种情况下细胞在膜上表达γ/δT细胞受体（TCR），而第二种情况下的细胞具有α/βTCR。遗传学研究表明，在病例1中TCRγ基因发生重排，β链基因构型为种系；TCR mRNA的γ链大小正常，而β链的mRNA被截断。病例2的TCR的β和γ基因发生重排，但仅表达α和βmRNA。与这些发现一致，TCR的δ链基因在病例1中发生重排，在病例2中缺失。病例1的细胞无细胞毒性活性，病例2的细胞毒性活性低；在后者中，与IL - 2或抗CD3单克隆抗体孵育后，裂解活性可上调。我们的研究表明，CD3 +、CD4 -、CD8 -淋巴细胞在LDGL患者中很少扩增。检测CD3 +、CD4 -、CD8 -、α/β +细胞的淋巴增殖性疾病可能有助于更好地表征这种新型淋巴细胞亚群。
