Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY.
Blood. 2024 Oct 31;144(18):1887-1897. doi: 10.1182/blood.2023021789.
The treatment of common nodal peripheral T-cell lymphomas (PTCLs), including PTCL, not otherwise specified (PTCL, NOS), anaplastic large-cell lymphomas, and T-follicular helper lymphomas, is evolving. These entities are currently treated similarly with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) or cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone (CHOEP) for CD30-negative diseases, or brentuximab vedotin plus cyclophosphamide, doxorubicin, and prednisone (CHP) for CD30-positive diseases, followed by consolidation with autologous stem cell transplantation in the first remission. Ongoing improvements in PTCL classification, identification of predictive biomarkers, and development of new targeted agents will lead to more specific therapies that address the unique biologic and clinical properties of each entity. For example, widespread efforts focused on molecular profiling of PTCL, NOS is likely to identify distinct subtypes that warrant different treatment approaches. New agents, such as EZH1/2 and JAK/STAT pathway inhibitors, have broadened treatment options for relapsed or refractory diseases. Furthermore, promising strategies for optimizing immune therapy for PTCL are currently under investigation and have the potential to significantly alter the therapeutic landscape. Ongoing frontline study designs incorporate an understanding of disease biology and drug sensitivities and are poised to evaluate whether newer-targeted agents should be incorporated into frontline settings for various disease entities. Although current treatment strategies lump most disease entities together, future treatments will include distinct strategies for each disease subtype that optimize therapy for individuals. This movement toward individualized therapy will ultimately lead to dramatic improvements in the prognosis of patients with PTCL.
常见结外外周 T 细胞淋巴瘤（PTCL）的治疗方法正在不断发展，包括未特指的外周 T 细胞淋巴瘤（PTCL，NOS）、间变大细胞淋巴瘤和 T 滤泡辅助细胞淋巴瘤。这些疾病目前采用环磷酰胺、多柔比星、长春新碱和泼尼松（CHOP）或环磷酰胺、多柔比星、长春新碱、依托泊苷和泼尼松（CHOEP）治疗 CD30 阴性疾病，或用 Brentuximab vedotin 联合环磷酰胺、多柔比星和泼尼松（CHP）治疗 CD30 阳性疾病，随后在首次缓解时进行自体干细胞移植巩固治疗。PTCL 分类、预测生物标志物的识别以及新靶向药物的开发方面的不断进展，将导致更具针对性的治疗方法，针对每种疾病实体的独特生物学和临床特征。例如，广泛开展的 PTCL，NOS 分子谱分析工作可能会确定不同的亚型，需要不同的治疗方法。新药物，如 EZH1/2 和 JAK/STAT 通路抑制剂，拓宽了复发或难治性疾病的治疗选择。此外，目前正在研究用于优化 PTCL 免疫治疗的有前途的策略，有可能显著改变治疗格局。正在进行的一线研究设计包括对疾病生物学和药物敏感性的理解，并准备评估是否应该将更新的靶向药物纳入各种疾病实体的一线治疗中。尽管目前的治疗策略将大多数疾病实体归为一类，但未来的治疗方法将包括针对每种疾病亚型的独特策略，为个体优化治疗。这种向个体化治疗的转变最终将显著改善 PTCL 患者的预后。
