Yoon Sang Eun, Kim Won Seog
Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Hematol Oncol. 2025 Jun;43 Suppl 2(Suppl 2):e70069. doi: 10.1002/hon.70069.
Peripheral T-cell lymphoma (PTCL) is a rare and heterogeneous group of diseases, with over 30 subtypes according to the International Consensus Classification of Mature Lymphoid Neoplasms (ICC) and World Health Organization Classification of Hematolymphoid Tumors (WHO-HEM) 2022. The classification complexity reflects the underlying genetic and biological diversity of PTCL. For decades, distinct PTCL subtypes have been uniformly treated with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or CHOP-like regimens originally developed for mainly B-cell lymphoma. Attempts to improve frontline CHOP-plus strategies have failed mainly due to toxicities and lack of biological rationale. Only the ECHELON-2 trial succeeded as more than 70% of patients had anaplastic large cell lymphoma (ALCL), where brentuximab vedotin (BV) is most effective. Looking ahead to 2025 and beyond, future treatment strategies for PTCL should be guided by a deeper understanding of its underlying biology rather than relying on empirical extrapolations from other lymphomas.
外周T细胞淋巴瘤（PTCL）是一组罕见且异质性的疾病，根据国际成熟淋巴细胞肿瘤共识分类（ICC）和世界卫生组织血液淋巴肿瘤分类（WHO-HEM）2022，有30多种亚型。分类的复杂性反映了PTCL潜在的遗传和生物学多样性。几十年来，不同的PTCL亚型一直统一采用CHOP（环磷酰胺、阿霉素、长春新碱和泼尼松）或最初主要为B细胞淋巴瘤开发的类似CHOP方案进行治疗。改善一线CHOP加方案的尝试主要因毒性和缺乏生物学依据而失败。只有ECHELON-2试验取得成功，因为超过70%的患者患有间变性大细胞淋巴瘤（ALCL），而本妥昔单抗（BV）在该疾病中最为有效。展望2025年及以后，PTCL的未来治疗策略应以对其潜在生物学的更深入理解为指导，而不是依赖于从其他淋巴瘤进行的经验性推断。
