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  • A heterogenous category of nodal and extranodal mature T-cell lymphomas that do not correspond to any of the specifically defined entities of mature T-cell lymphoma in the 2017 WHO classification. Excluded from this category are tumors with a T follicular helper (TFH) cell phenotype. Variants include lymphoepithelioid lymphoma (Lennert lymphoma) and primary EBV-positive nodal T-cell or NK-cell lymphoma. The follicular variant included in the peripheral T-cell lymphomas, not otherwise specified, in the 2008 edition of the WHO classification has been moved to the category of angioimmunoblastic T-cell lymphoma and other nodal lymphomas of T follicular helper cell origin in the 2017 WHO update. The same is true for a proportion of cases previously designated as the T-zone variant, because they usually have a TFH-cell phenotype. (WHO 2017)
  • This type of lymphoma is not frequently seen in the western hemisphere. Clinically, with the exception of anaplastic large cell lymphoma, mature T- and NK-cell lymphomas are among the most aggressive of all hematopoietic neoplasms. Representative disease entities include mycosis fungoides, angioimmunoblastic T-cell lymphoma, hepatosplenic T-cell lymphoma, and anaplastic large cell lymphoma.
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