from Section V - Mature Lymphoid Neoplasms
Published online by Cambridge University Press: 25 January 2024
Peripheral (mature) T-cell lymphomas (PTCLs) are much less common than B-cell lymphomas. Derived from post-thymic T-cells, PTCLs generally arise in lymphoid tissues “peripheral” to the thymus, such as the lymph nodes, spleen, gastrointestinal tract, and skin. Because natural killer (NK) cells and T-cells arise from a common progenitor cell and have some overlapping properties, NK-cell lymphomas are considered together with PTCLs in the World Health Organization (WHO) classification system [1].
PTCLs occur most frequently in adults and less frequently in children and adolescents. They comprise about 10–15% of all non-Hodgkin lymphomas (NHLs) in pediatric age groups [2]. NK-cell neoplasms are even rarer. Unlike adults, where there is a broad spectrum of T-cell neoplasms, the most common type of PTCL in pediatric patients is anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma, with other subtypes of PTCL (0.9%) being observed much less frequently [3].
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