Falini B, Flenghi L, Fagioli M, Martelli M F, Pileri S, Grignani F, Beltrami A, Novero D, Pelicci P G
Institute of Clinical Medicine, University of Perugia, Italy.
Blood. 1989 Nov 15;74(7):2501-7.
Three cases of T-lymphoblastic lymphomas (T-LL) expressing the T cell antigen receptor gamma delta (TCR gamma delta) are reported. All of them were CD3+/beta F1-/TCR delta 1+. Moreover, neoplastic cells reacted with the delta TCS1 monoclonal antibody (MoAb) which binds to the non-disulfide-linked form of the TCR gamma delta, but not with the BB3 MoAb which recognizes the disulfide-linked form of the TCR gamma delta. All cases showed a stage II cortical phenotype, eg, TdT+/CD1+/CD3+/CD5+/CD7+; two of them coexpressed CD4/CD8, while the other was CD4+/CD8-. Two cases were positive for CALLA and CD25. Immunogenotypic analysis showed evidence of T beta and C gamma 2 gene rearrangements in all three cases and immunoglobulin (Ig) gene rearrangements in two cases. Two patients presented with an anterior mediastinal mass and the third with a solitary inguinal lymphadenopathy. We suggest that these cases of TCR gamma delta+ T-LL may be derived from the small population (approximately 0.5%) of CD3+ cortical thymocytes which, in the normal human thymus, express the delta TCS1-reactive, non-disulfide-linked form of the TCR gamma delta.
报告了3例表达T细胞抗原受体γδ（TCRγδ）的T淋巴细胞淋巴瘤（T-LL）。所有病例均为CD3+/βF1-/TCRδ1+。此外，肿瘤细胞与结合TCRγδ非二硫键连接形式的δTCS1单克隆抗体（MoAb）发生反应，但不与识别TCRγδ二硫键连接形式的BB3 MoAb发生反应。所有病例均表现为II期皮质表型，例如TdT+/CD1+/CD3+/CD5+/CD7+；其中2例共表达CD4/CD8，另1例为CD4+/CD8-。2例CALLA和CD25呈阳性。免疫基因型分析显示，所有3例均有Tβ和Cγ2基因重排，2例有免疫球蛋白（Ig）基因重排。2例患者表现为前纵隔肿块，第3例表现为孤立性腹股沟淋巴结病。我们认为，这些TCRγδ+T-LL病例可能来源于正常人类胸腺中表达δTCS1反应性、非二硫键连接形式TCRγδ的一小部分（约0.5%）CD3+皮质胸腺细胞。
