Foster Cheryl, Kuruvilla John
Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, 6-424 700 University Avenue, Toronto, ON, M5G 1Z5, Canada.
Department of Medicine, University of Toronto, 1 King's College Cir, Toronto, ON, M5S 1A8, Canada.
F1000Res. 2020 Sep 4;9. doi: 10.12688/f1000research.22257.1. eCollection 2020.
Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of rare and aggressive non-Hodgkin's lymphomas. Clinical staging, prognostic scoring, and initial treatment strategies have historically been based on paradigms developed in B-cell lymphomas. Despite primary treatment protocols that are typically anthracycline-based and frequently involve consolidative autologous stem cell transplantation in first remission, many patients develop disease progression. There remains a high unmet medical need for improved treatment strategies in the relapsed or refractory setting. Salvage chemotherapy and stem cell transplantation in those who are suitable has traditionally been the accepted approach, but this remains a minority of the total patient population. As increasing knowledge is gleaned regarding the biological heterogeneity within the various PTCL subtypes, newer targeted agents have been developed, studied, and approved in this small, heterogeneous population of relapsed or refractory disease. Given its success and tolerability in this pretreated population, brentuximab vedotin, an anti-CD30 antibody drug conjugate, was brought earlier in the disease course and is a model for advances in the targeted treatment of PTCL. As others undergo further development in the relapsed setting and successes are brought earlier in the disease course, the outcome for PTCL patients is likely to improve. However, innovative clinical trial designs are crucial for the assessment of targeted agents in this highly heterogeneous population. This review explores the current treatment environment for patients with relapsed and refractory PTCL, including newer strategies such as targeted agents and immunotherapy.
外周T细胞淋巴瘤（PTCL）是一组异质性的罕见且侵袭性非霍奇金淋巴瘤。临床分期、预后评分和初始治疗策略历来基于B细胞淋巴瘤所建立的模式。尽管初始治疗方案通常以蒽环类药物为基础，且在首次缓解时经常涉及巩固性自体干细胞移植，但许多患者仍会出现疾病进展。对于复发或难治性患者，改善治疗策略的医疗需求仍未得到满足。传统上，适合的患者采用挽救性化疗和干细胞移植，但这仍然只占总患者群体的少数。随着对各种PTCL亚型内生物学异质性的了解不断增加，已经开发、研究并在这一小部分异质性复发或难治性疾病患者中批准了更新的靶向药物。鉴于抗CD30抗体药物偶联物brentuximab vedotin在这一预处理人群中的成功和耐受性，它在疾病进程中更早被应用，是PTCL靶向治疗进展的一个典范。随着其他药物在复发情况下进一步发展，并在疾病进程中更早取得成功，PTCL患者的预后可能会得到改善。然而，创新的临床试验设计对于评估这一高度异质性人群中的靶向药物至关重要。本综述探讨了复发和难治性PTCL患者当前的治疗环境，包括靶向药物和免疫疗法等新策略。
