Qiu Lianqun, Cho Jeong Hee, Jelloul Fatima Zahra, Vega Francisco
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, Texas.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, Texas; UT Health Graduate School of Biomedical Sciences, Houston, Texas.
Clin Lymphoma Myeloma Leuk. 2022 May;22(5):287-296. doi: 10.1016/j.clml.2021.10.006. Epub 2021 Oct 15.
Peripheral T-cell lymphomas (PTCLs) are a heterogeneous and often clinically aggressive group of neoplasms derived from mature post-thymic T-lymphocytes. These neoplasms are rare and usually diagnostically challenging. Our understanding of the pathogenesis of PTCL is increasing and this improved knowledge is leading us to better molecular characterization, more objective and accurate diagnostic criteria, more effective risk assessment, and potentially better treatments. The focus of this paper is to present a brief overview of the current pathology criteria and molecular and genetic features of nodal peripheral T-cell lymphomas focusing on distinct genetically and molecularly defined subgroups that are being recognized within each major nodal PTCL category. It is expected that the molecular stratification will improve the diagnosis and will provide novel therapeutic opportunities (biomarker-driven and targeted therapies) that might benefit and change the outcomes of patients with these neoplasms.
外周T细胞淋巴瘤(PTCL)是一组异质性的肿瘤,通常具有临床侵袭性,起源于成熟的胸腺后T淋巴细胞。这些肿瘤较为罕见,诊断通常具有挑战性。我们对PTCL发病机制的认识不断增加,这种知识的进步正引导我们实现更好的分子特征描述、更客观准确的诊断标准、更有效的风险评估以及可能更好的治疗方法。本文的重点是简要概述淋巴结外周T细胞淋巴瘤的当前病理学标准以及分子和遗传特征,重点关注在每个主要的淋巴结PTCL类别中正在被认识到的不同的基因和分子定义的亚组。预计分子分层将改善诊断,并提供可能使这些肿瘤患者受益并改变其预后的新治疗机会(生物标志物驱动和靶向治疗)。