Peripheral T-Cell Lymphoma NOS: Current Management and Emerging Therapeutic Strategies

Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of lymphoproliferative disorders arising from mature T-cells and account for approximately 10% of all non-Hodgkin lymphomas. PTCL not otherwise specified (PTCL-NOS) is the most common subtype and is considered a diagnosis of exclusion after other PTCL subtypes have been ruled out. Therefore, PTCL-NOS exhibits extreme heterogeneity in its morphologic features, molecular characteristics, and clinical manifestations. Prognosis in PTCL is dismal with a 5-year overall survival (OS) of 20–30%. Diagnosis can be challenging, and efforts to improve risk stratification and optimize therapeutic management continue to evolve. This review systematically summarizes recent advances in the understanding of the epidemiology, classification, histopathology, diagnosis, and management of PTCL-NOS. We discuss emerging therapeutic options and future directions aimed at improving outcomes for this aggressive disease.