Mature T- en NK-cel neoplasma
WHO 2022: T-cell and NK-cell lymphoid proliferations and lymphomas
Tumour-like lesions with T-cell predominance
Kikuchi-Fujimoto disease
Indolent T-lymphoblastic proliferation
Autoimmune lymphoproliferative syndrome (ALPS)
Precursor T-cell neoplasms
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
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.