SOHO State of the Art Updates and Next Questions | Peripheral T-Cell Lymphoma: Current Landscape and Emerging...

Peripheral T-cell lymphomas (PTCLs) comprise a rare, heterogeneous group of mature T-cell and NK-cell neoplasms, representing approximately 10% to 15% of all non-Hodgkin lymphomas. Despite advances in disease classification and biology, clinical outcomes remain poor for most subtypes other than anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL). This review synthesizes current understanding of nodal PTCL biology-including T-cell follicular helper (TFH)-derived lymphomas, PTCL-NOS, and systemic ALCL. The integration of molecular profiling has deepened our understanding of disease heterogeneity, identifying recurrent epigenetic and signaling pathway alterations that now inform rational, targeted strategies. Brentuximab vedotin-based regimens have reshaped frontline therapy for CD30-positive disease, while ongoing studies are exploring the role of novel therapies including epigenetic modulators, phosphatidylinositol 3-kinase (PI3K) and JAK inhibitors, and immune-based therapies across the PTCL spectrum. Future progress will depend on biomarker-driven clinical trials, refined patient selection, and the incorporation of genomic and immune signatures to personalize therapy.