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儿童T细胞系白血病/淋巴瘤中T细胞β链和γ链基因的伴随重排

Concomitant rearrangements of T-cell beta- and gamma-chain genes in childhood T-lineage leukemia/lymphoma.

作者信息

Ha-Kawa K, Yumura K, Hara J, Ishihara S, Yabuuchi H

出版信息

Leuk Res. 1987;11(8):739-45. doi: 10.1016/0145-2126(87)90011-7.

Abstract

Similar to the immunoglobulin (Ig) gene rearrangements in B-lineage cells, identification of T-cell receptor (TCR) gene rearrangements is a novel clonal marker and necessary to establish a T-cell lineage. The function of T-cell gamma-chain (T gamma) gene is still unknown, but because of its shared properties with T-cell alpha-chain (T alpha) and T beta genes, we analysed T gamma gene organization in 10 patients with T-lineage leukemia/lymphoma as well as in non-T lineage leukemias. All 10 cases of T-lineage leukemia/lymphoma, whose phenotypes were different, demonstrated T gamma gene rearrangements as well as T beta gene rearrangements. In contrast, among the non-T-lineage leukemias, the emergence of T beta and/or T gamma gene rearrangements was varied. Based on these findings, concomitant rearrangements of T beta and T gamma genes are characteristic in childhood T-lineage leukemia/lymphoma regardless of their phenotypic differences. Furthermore, no obvious developmental hierarchy was observed between T beta and T gamma gene arrangements in these leukemia/lymphoma cells.

摘要

与B淋巴细胞系细胞中的免疫球蛋白(Ig)基因重排类似,T细胞受体(TCR)基因重排的鉴定是一种新的克隆标志物,也是确定T细胞系所必需的。T细胞γ链(Tγ)基因的功能尚不清楚,但由于其与T细胞α链(Tα)和Tβ基因具有共同特性,我们分析了10例T细胞系白血病/淋巴瘤患者以及非T细胞系白血病中Tγ基因的组织情况。10例T细胞系白血病/淋巴瘤患者,其表型各异,均显示出Tγ基因重排以及Tβ基因重排。相比之下,在非T细胞系白血病中,Tβ和/或Tγ基因重排的出现情况各不相同。基于这些发现,无论其表型差异如何,Tβ和Tγ基因的同时重排在儿童T细胞系白血病/淋巴瘤中具有特征性。此外,在这些白血病/淋巴瘤细胞中,未观察到Tβ和Tγ基因排列之间存在明显的发育层次。

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