T-cell and natural killer T-cell lymphomas

Last revised by Frank Gaillard on 19 Mar 2023

T-cell and natural killer T-cell lymphomas are a large group of rare and aggressive lymphoid neoplasms derived from T or natural killer (NK) cells. In the 5th edition (2022) of the WHO classification of haematolymphoid tumors, thirty-six entities are recognized 2. These lymphomas can involve various organs, including the lymph nodes and spleen and extranodal sites including skin, gastrointestinal tract and central nervous system 1,2.

T-cell and NK/T-cell lymphomas account for about 10-15% of all non-Hodgkin lymphomas worldwide. These lymphomas are more common in Asia and Latin America than in North America and Europe. They typically affect adults, with a slight male predominance 1,3.

The clinical presentation of T-cell and NK/T-cell lymphomas varies depending on the site of involvement. Patients may present with lymphadenopathy, fever, night sweats, weight loss, or B-symptoms. Skin involvement can present as a rash or ulceration, and gastrointestinal involvement can cause abdominal pain, diarrhea, or bleeding. Central nervous system involvement has a non-specific presentation including headaches, focal neurological deficits, seizures and features of increased intracranial pressure 1. Involvement of the nasal cavity (as in Nasal T-cell/NK-cell lymphoma) typically presents with nasal blockage, bleeding or discharge or constitutional symptoms 4.

Radiographic features of T-cell and NK/T-cell lymphomas vary depending on the subtype and site of involvement. Although appearances are similar to more common B-cell lymphoma, as a general rule necrosis is more common 1-4.

Treatment of T-cell and NK/T-cell lymphomas depends on the subtype and stage of the disease. Chemotherapy is the mainstay of treatment, with additional options including radiation therapy, stem cell transplantation, and immunotherapy.

The prognosis of T-cell and NK/T-cell lymphomas is generally poor.

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