文章：

TCR-beta F1和/或EZRIN表达的缺失与淋巴结周围t细胞淋巴瘤的不良预后相关

Loss of TCR-beta F1 and/or EZRIN expression is associated with unfavorable prognosis in nodal peripheral T-cell lymphomas

原文发布日期：2013-04-19

DOI: 10.1038/bcj.2013.10

类型: Original Article

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英文摘要：

Nodal peripheral T-cell lymphoma (nodal PTCL) has an unfavorable prognosis, and specific pathogenic alterations have not been fully identified. The biological and clinical relevance of the expression of CD30/T-cell receptor (TCR) genes is a topic under active investigation. One-hundred and ninety-three consecutive nodal PTCLs (89 angioimmunoblastic T-cell lymphomas (AITL) and 104 PTCL-unspecified (PTCL-not otherwise specified (NOS)) cases) were analyzed for the immunohistochemical expression of 19 molecules, involving TCR/CD30 pathways and the associations with standard prognostic indices. Mutually exclusive expression was found between CD3 and TCR-beta F1 with CD30 expression. Taking all PTCL cases together, logistic regression identified a biological score (BS) including TCR molecules (TCR-beta F1 and EZRIN) that separates two subgroups of patients with a median survival of 34.57 and 5.20 months (P<0.001). Multivariate analysis identified BS and the prognostic index for PTCL (PIT) score as independent prognostic factors. This BS maintained its significance in multivariate analysis only for the PTCL-NOS subgroup of tumors. In AITL cases, only a high level of ki67 expression was related to prognosis. A BS including molecules involved in the TCR signaling pathway proved to be an independent prognostic factor of poor outcome in a multivariate analysis, specifically in PTCL-NOS patients. Nevertheless, validation in an independent series of homogeneously treated PTCL patients is required to confirm these data.

摘要翻译： 

结节性外周T细胞淋巴瘤（nodal PTCL）预后不良，且其特异性致病机制尚未完全明确。目前，CD30/T细胞受体（TCR）基因表达的生物学和临床意义正处于深入研究阶段。本研究连续纳入193例结节性PTCL（包括89例血管免疫母细胞性T细胞淋巴瘤（AITL）及104例非特指型外周T细胞淋巴瘤（PTCL-NOS）），通过免疫组织化学方法检测19种涉及TCR/CD30通路的分子表达，并分析其与标准预后指标的关联。研究发现CD3、TCR-βF1与CD30表达呈互斥关系。通过逻辑回归分析所有PTCL病例，构建了包含TCR分子（TCR-βF1和EZRIN）的生物学评分（BS），该评分可将患者分为中位生存期分别为34.57个月和5.20个月的两个亚组（P<0.001）。多变量分析确认BS和PTCL预后指数（PIT）评分均为独立预后因素。该BS仅在PTCL-NOS亚组肿瘤的多变量分析中保持显著预后价值。而在AITL病例中，仅Ki67高表达与预后相关。本研究证实，包含TCR信号通路相关分子的BS在多变量分析中是PTCL-NOS患者不良预后的独立预测因素，但尚需在经统一治疗的独立PTCL患者队列中进行验证以确认上述结果。

原文链接：

Loss of TCR-beta F1 and/or EZRIN expression is associated with unfavorable prognosis in nodal peripheral T-cell lymphomas