Mature T- en NK-cel neoplasma

WHO 2022: T-cell and NK-cell lymphoid proliferations and lymphomas


  • Mature T-cell and NK-cell neoplasms (9 families)

    • Mature T-cell and NK-cell leukaemias

      • T-prolymphocytic leukaemia

      • T-large granular lymphocytic leukaemia

      • NK-large granular lymphocytic leukaemia

      • Adult T-cell leukaemia/lymphoma

      • Sezary syndrome

      • Aggressive NK-cell leukaemia

    • Primary cutaneous T-cell lymphomas

      • Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder

      • Primary cutaneous acral CD8-positive lymphoproliferative disorder

      • Mycosis fungoides

      • Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Lymphomatoid papulosis

      • Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Primary cutaneous anaplastic large cell lymphoma

      • Subcutaneous panniculitis-like T-cell lymphoma

      • Primary cutaneous gamma/delta T-cell lymphoma

      • Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma

      • Primary cutaneous peripheral T-cell lymphoma, NOS

    • Intestinal T-cell and NK-cell lymphoid proliferations and lymphomas

      • Indolent T-cell lymphoma of the gastrointestinal tract

      • Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract

      • Enteropathy-associated T-cell lymphoma

      • Monomorphic epitheliotropic intestinal T-cell lymphoma

      • Intestinal T-cell lymphoma, NOS

    • Hepatosplenic T-cell lymphoma

    • Anaplastic large cell lymphoma

      • ALK-positive anaplastic large cell lymphoma

      • ALK-negative anaplastic large cell lymphoma

      • Breast implant-associated anaplastic large cell lymphoma

    • Nodal T-follicular helper (TFH) cell lymphoma

      • Nodal TFH cell lymphoma, angioimmunoblastic-type

      • Nodal TFH cell lymphoma, follicular-type

      • Nodal TFH cell lymphoma, NOS

    • Other peripheral T-cell lymphomas

    • EBV-positive NK/T-cell lymphomas

      • EBV-positive nodal T- and NK-cell lymphoma

      • Extranodal NK/T-cell lymphoma

    • EBV-positive T- and NK-cell lymphoid proliferations and lymphomas of childhood

      • Severe mosquito bite allergy

      • Hydroa vacciniforme lymphoproliferative disorder

      • Systemic chronic active EBV disease

      • Systemic EBV-positive T-cell lymphoma of childhood


The mature T-cell and NK-cell neoplasms are grouped into 9 families based on diverse concepts: cell of origin/differentiation state, clinical scenario, disease localization, and cytomorphology. While most T- or NK-cell neoplasms can be assigned to the respective T- or NK-cell lineage, they are not separated as two

categories in WHO-HAEM5 because some entities comprise a spectrum of tumours of NK, T, hybrid or indeterminate phenotype, such as in extranodal NK/T-cell lymphoma, EBV+ nodal T- and NKcell lymphoma, chronic active EBV disease and severe mosquito

bite allergy. In other instances, distinction between T- and NK-cell origin may be unclear or difficult to determine.