Historical advances in the care of patients with non‐Hodgkin lymphoma (NHL) have been restricted largely to patients with B‐cell lymphoma. The peripheral T‐cell lymphomas (PTCLs), which are rare and heterogeneous in nature, have yet to experience the same degree of improvement in outcome over the past 20 to 30 years. It is estimated that there are approximately 80,000 and 14,000 cases, respectively, of NHL and Hodgkin lymphoma per year in the United States. As a subgroup of NHL, the PTCLs account for 6% to 10% of all cases of NHL, making them exceedingly rare. In addition, the World Health Organization 2017 classification describes 29 distinct subtypes of PTCL. This intrinsic diversity, coupled with its rarity, has stymied progress in the disease. In addition, most subtypes carry an inferior prognosis compared with their B‐cell counterparts, an outcome largely attributed to the fact that most treatment paradigms for patients with PTCL have been derived from B‐cell neoplasms, a radically different disease. In fact, the first drug ever approved for patients with PTCL was approved only a decade ago. The plethora of recent drug approvals in PTCL, coupled with a deeper understanding of the molecular pathogenesis of the disease, has stimulated the field to pursue new avenues of research that are now largely predicated on the development of novel, targeted small molecules, which include a host of epigenetic modifiers and biologics. There is an expectation these advances may begin to favorably challenge the chemotherapy paradigms that have been used in the T‐cell malignancies.

中文翻译：

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成熟 T 细胞淋巴瘤 (MTCL) 生物学和管理的快速变化

非霍奇金淋巴瘤 (NHL) 患者治疗的历史进展主要限于 B 细胞淋巴瘤患者。外周 T 细胞淋巴瘤 (PTCL) 在性质上是罕见且异质的，但在过去 20 至 30 年中尚未经历相同程度的结果改善。据估计，美国每年分别有大约 80,000 和 14,000 例 NHL 和霍奇金淋巴瘤病例。作为 NHL 的一个亚组，PTCL 占所有 NHL 病例的 6% 至 10%，使其极为罕见。此外，世界卫生组织 2017 年分类描述了 29 种不同的 PTCL 亚型。这种内在的多样性，加上它的稀有性，阻碍了这种疾病的进展。此外，与 B 细胞亚型相比，大多数亚型的预后较差，这一结果在很大程度上归因于这样一个事实，即大多数 PTCL 患者的治疗范式都源自 B 细胞肿瘤，这是一种完全不同的疾病。事实上，第一种被批准用于 PTCL 患者的药物仅在十年前才被批准。PTCL 最近获得的大量药物批准，加上对该疾病分子发病机制的更深入了解，刺激了该领域寻求新的研究途径，这些途径现在主要依赖于开发新型靶向小分子，其中包括大量的表观遗传修饰剂和生物制剂。预计这些进展可能会开始积极挑战已用于 T 细胞恶性肿瘤的化疗范式。