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Aggressive NK- and T-cell lymphomas have a fairly poor prognosis.
Lymphomas arising from mature T-cells are categorized under the general term peripheral T-cell lymphoma (PTCL), distinguishing them from lymphomas that arise from immature T-cells of lymphoblastic lymphoma.
In 2016, the World Health Organization categorized 27 different PTCL subtypes, with the most common 6 subtypes comprising >75% of cases. These include PTCL-Not Otherwise Specified (PTCL-NOS), anaplastic lymphoma kinase (ALK+/ALK-) anaplastic large cell lymphoma (ALCL), angioimmunoblastic T-cell lymphoma (AITL), NK/T-cell lymphoma, and adult T-cell leukemia/lymphoma (ATLL).
Diagnosis can be challenging and is based on clinical features and presentation, as well as surface markers and genetic factors.
PTCL-NOS refers to a group of diseases that do not fit into any of the other PTCL subtypes, and accounts for ~20% of all T-cell lymphomas. ALCL is characterized by CD30+ anaplastic large cells, of which there is 3 types – systemic, primary cutaneous and breast-implant associated ALCL.
Systemic ALCL can present as ALK+ or ALK-, which has implications for prognosis and treatment. ALK+ ALCL is more commonly seen in younger patients, and has a better prognosis than ALK- ALCL.
Primary cutaneous ALCL has an excellent prognosis, and treatment is primarily skin-directed.
AITL is a rare, aggressive T-cell subtype, and there is evidence it develops from an ongoing immune response to latent viral infections, such as Epstein-Barr virus. Most diagnosed are middle-aged or elderly and are diagnosed at an advanced stage.
ATLL is another rare and often aggressive T-cell lymphoma found in the blood, lymph nodes, skin or multiple areas of the body. It is linked to human T-cell lymphotropic virus type 1 (HTLV-1) infection.
It requires urgent treatment in most patients and stem cell transplantation is frequently needed for cure.
The CHOP-based chemotherapy regimen has remained the standard of treatment for most PTCLs in recent years. There is no current consensus on the standard treatment option for relapsed/refractory PTCL; however, newer treatments are now available for PTCL, including antibody-drug conjugates, antifolates and HDAC inhibitors.
NK/T-cell lymphoma usually known as extranodal NK/T-cell lymphoma nasal type is a rare type of lymphoma with a poor prognosis. The disease is more common in Asian and Latin American countries and is strongly associated with the Epstein Barr virus, predominantly affecting the nose and face.
The immunophenotype of lymphoma cells reflects NK cells mostly but can reflect T-cells as well. Disease is able to spread outside the original site where it quickly progresses and leads to rapid deterioration.
Limited disease involves 3-4 courses of chemotherapy usually including anthracycline and irradiation which has an overall 5 year survival rate of less than 50%. Advanced disease generally has a poorer outcome even with chemotherapy.