T-cell large granular lymphocyte leukemia is characterized by massive TCRBV-restricted clonal CD8 expansion and a generalized overexpression of the effector cell marker CD57.
T-cell large granular lymphocyte leukemia is characterized by massive TCRBV-restricted clonal CD8 expansion and a generalized overexpression of the effector cell marker CD57.
复制标题
DOI：
10.1038/sj.thj.6200212
复制
发表时间：
2003-01-01
期刊：
影响因子：
--
通讯作者：
Barrett, A John
中科院分区：
文献类型：
--
作者：
Melenhorst, J Joseph;Eniafe, Rhoda;Barrett, A John
引言：大颗粒淋巴细胞白血病（LGL）是一种表达CD 57活化标志物的CD 8 + T细胞的克隆性淋巴组织增生性疾病。然而，尚不清楚CD 57+群体是否代表LGL克隆。我们以前证明，克隆可以在CD 8 + CD 57+和CD 8 + CD 57-细胞中发现，表明LGL克隆也驻留在CD 57- fraction.MATERIALS和METHODS：在这里，我们量化的程度克隆CD 8扩增在LGL使用T细胞受体V β（TCRBV）特异性单克隆抗体，并确定是否CD 4人口也包含偏斜。此外，显性TCRBV群体的克隆状态进行了评估，使用T细胞受体-γ（TCRG）PCR基因组DNA.Results：我们表明，在LGL中的优势TCRBV含有CD 57+和CD 57-细胞。通过TCRG PCR对显性TCRBV的CD 8 + CD 57+和CD 8 + CD 57-亚组分的分子分析表明，这两种组分确实是克隆的，并且该克隆在显性TCRBV阴性群体中不存在。此外，我们表明，CD 57过表达并不局限于LGL克隆，但在CD 8细胞的LGL patients.CONCLUSION的一般现象：因此，我们得出结论，LGL的主要特点是克隆扩增的CD 8细胞，与此同时上调CD 57对这个克隆和无关的细胞。
INTRODUCTION: Large granular lymphocyte leukemia (LGL) is a clonal lymphoproliferative disease of CD8+ T cells expressing the CD57 activation marker. It is, however, unknown whether the CD57+ population represents the LGL clone or not. We previously demonstrated that the clone can be found in both CD8+CD57+ and CD8+CD57- cells, indicating that the LGL clone also resides in the CD57- fraction.MATERIALS AND METHODS: Here, we quantified the extent of the clonal CD8 expansion in LGL using T-cell receptor Vbeta (TCRBV)-specific monoclonal antibodies, and determined whether the CD4 population also contained skews. Furthermore, dominant TCRBV populations were assessed for clonal status using T-cell receptor-gamma (TCRG) PCR on genomic DNA.RESULTS: We show that the dominant TCRBV in LGL contains CD57+ and CD57- cells. Molecular analysis of CD8+CD57+ and CD8+CD57- subfractions of the dominant TCRBV by TCRG PCR demonstrates that indeed both fractions are clonal, and that the clone is absent from the dominant TCRBV-negative population. Furthermore, we show that CD57 overexpression is not restricted to the LGL clone, but a general phenomenon in CD8 cells of LGL patients.CONCLUSION: We therefore conclude that the primary characteristic of LGL is a clonal expansion of CD8 cells, with a concomitant upregulation of CD57 on this clone and uninvolved cells.