Abstract
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.