文章：

外周T细胞淋巴瘤：从实验室到临床

Peripheral T cell lymphomas: from the bench to the clinic

原文发布日期：2020-04-06

DOI: 10.1038/s41568-020-0247-0

类型: Review Article

开放获取: 否

英文摘要：

Peripheral T cell lymphomas (PTCLs) are a heterogeneous group of orphan neoplasms. Despite the introduction of anthracycline-based chemotherapy protocols, with or without autologous haematopoietic transplantation and a plethora of new agents, the progression-free survival of patients with PTCLs needs to be improved. The rarity of these neoplasms, the limited knowledge of their driving defects and the lack of experimental models have impaired clinical successes. This scenario is now rapidly changing with the discovery of a spectrum of genomic defects that hijack essential signalling pathways and foster T cell transformation. This knowledge has led to new genomic-based stratifications, which are being used to establish objective diagnostic criteria, more effective risk assessment and target-based interventions. The integration of genomic and functional data has provided the basis for targeted therapies and immunological approaches that underlie individual tumour vulnerabilities. Fortunately, novel therapeutic strategies can now be rapidly tested in preclinical models and effectively translated to the clinic by means of well-designed clinical trials. We believe that by combining new targeted agents with immune regulators and chimeric antigen receptor-expressing natural killer and T cells, the overall survival of patients with PTCLs will dramatically increase.

摘要翻译： 

外周T细胞淋巴瘤（PTCL）是一组异质性的罕见肿瘤。尽管已引入蒽环类化疗方案（联合或不联合自体造血干细胞移植）及多种新型药物，但PTCL患者的无进展生存期仍有待改善。这类肿瘤的罕见性、对其驱动缺陷认知的有限性以及实验模型的缺乏阻碍了临床成功。随着一系列劫持关键信号通路并促进T细胞转化的基因组缺陷的发现，这一现状正迅速改变。这些认知催生了基于基因组的新分层方法，现正用于建立客观诊断标准、更有效的风险评估及靶向干预措施。基因组与功能数据的整合为靶向治疗和免疫疗法奠定了基础，这些方法针对个体肿瘤的脆弱性。值得庆幸的是，新型治疗策略如今可在临床前模型中快速验证，并通过精心设计的临床试验有效转化为临床实践。我们相信，通过将新型靶向药物与免疫调节剂、表达嵌合抗原受体的自然杀伤细胞及T细胞相结合，PTCL患者的总生存期将显著延长。

原文链接：

Peripheral T cell lymphomas: from the bench to the clinic