Kanavaros P, Farcet J P, Gaulard P, Haioun C, Divine M, Le Couedic J P, Lefranc M P, Reyes F
Institut Nationale de la Santé et de la Recherche Médicale U 91, Hôpital Henri Mondor, Créteil, France.
J Clin Invest. 1991 Feb;87(2):666-72. doi: 10.1172/JCI115044.
Recombinative events of the T cell antigen receptor (TCR) delta-chain gene were studied in 37 cases of peripheral T cell lymphoma (PTCL) and related to their clinical presentation and the expression of the alpha beta or gamma delta heterodimers as determined by immunostaining of frozen tissue samples. There were 22 cases of alpha beta, 5 cases of gamma delta, and 10 cases of silent TCR expressing neither the alpha beta nor gamma delta TCR. 5 different probes were used to examine the delta locus. The 22 cases of alpha beta PTCL displayed biallelic and monoallelic deletions; a monoallelic V delta 1 J delta 1 rearrangement was observed in 1 case and a monoallelic germ line configuration in 7 cases. The 5 cases of gamma delta PTCL displayed biallelic rearrangements: the productive rearrangements could be ascribed to V delta 1J delta 1 joining in 3 cases and VJ delta 1 joining in 2 cases according to the combined pattern of DNA hybridization with the appropriate probes and of cell reactivity with the TCR delta-1, delta TCS-1, and anti-V delta 2 monoclonal antibodies. In the VJ delta 1 joining, the rearranged V segments were located between V delta 1 and V delta 2. Interestingly, in the third group of 10 cases of silent PTCL, 5 cases were found to have a TCR gene configuration identical to that in the TCR alpha beta PTCL, as demonstrated by biallelic delta gene deletion. These 5 cases were CD3 positive. The 5 remaining cases showed a monoallelic delta gene rearrangement with a monoallelic germ line configuration in 4 and a monoallelic deletion in 1. Four of these cases were CD3 negative, which was consistent with an immature genotype the TCR commitent of which could not be ascertained. Finally, TCR gamma delta PTCL consisted of a distinct clinical morphological and molecular entity whereas TCR alpha beta and silent PTCL had a similar presentation.
我们研究了37例外周T细胞淋巴瘤（PTCL）中T细胞抗原受体（TCR）δ链基因的重组事件，并将其与临床表现以及通过冷冻组织样本免疫染色确定的αβ或γδ异二聚体的表达情况相关联。其中αβ型22例，γδ型5例，10例为沉默型TCR，既不表达αβ也不表达γδTCR。使用5种不同的探针检测δ基因座。22例αβ型PTCL表现为双等位基因和单等位基因缺失；1例观察到单等位基因Vδ1Jδ1重排，7例为单等位基因种系构型。5例γδ型PTCL表现为双等位基因重排：根据与适当探针的DNA杂交组合模式以及细胞与TCRδ-1、δTCS-1和抗Vδ2单克隆抗体的反应性，3例的有效重排可归因于Vδ1Jδ1连接，2例归因于VJδ1连接。在VJδ1连接中，重排的V片段位于Vδ1和Vδ2之间。有趣的是，在第三组10例沉默型PTCL中，5例被发现具有与TCRαβ型PTCL相同的TCR基因构型，双等位基因δ基因缺失证明了这一点。这5例CD3呈阳性。其余5例显示单等位基因δ基因重排，4例为单等位基因种系构型，1例为单等位基因缺失。其中4例CD3呈阴性，这与一种无法确定TCR定向的未成熟基因型一致。最后，TCRγδ型PTCL由一个独特的临床形态学和分子实体组成，而TCRαβ型和沉默型PTCL具有相似的表现。
