Abstract:
The peripheral T/NK cell lymphomas (PT/NKCLs) encompass a heterogeneous group of malignant lymphoid diseases, which derived from mature T-cells and NK cells. PT/NKCLs are rare, and its epidemiology and pathological subtypes showed significant geographic and ethnic differences. PT/NKCLs, especially NK/T cell lymphoma (nasal type), are relatively common in Asian countries. The use of an anthracycline-containing regimen is not associated with an improved outcome in most subtypes of PT/NKCLs, but is associated with an improved outcome in anaplastic large-cell lymphoma, ALK positive. High-dosage chemotherapy combined with stem cell transplantation is a considerable choice for newly diagnosed high-risk or refractory/relapsed PT/NKCLs because of its generally poor clinical outcome. Advances in new drugs have lagged behind DLBCL because of the disease rarity of PT/NKCL and biological heterogeneity. Nevertheless, new chemotherapeutic drugs, such as pralatrexate and bendamustine, and targeted drugs, such as alemtuzumab, brentuximab vedotin, HDAC inhibitors, bortezomib, and lenalidomide, are recently emerging effective therapies in this area. We confidently believe that the era of PT/NKCLs is coming.