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. 2022 Jun 22;36(7):1720–1748. doi: 10.1038/s41375-022-01620-2

Table 2.

WHO Classification of Haematolymphoid Tumours, 5th edition: T-cell and NK-cell lymphoid proliferations and lymphomas.

WHO Classification, 5th edition WHO Classification, revised 4th edition
Tumour-like lesions with T-cell predominance
Kikuchi-Fujimoto disease Not previously included
Indolent T-lymphoblastic proliferation Not previously included
Autoimmune lymphoproliferative syndrome Not previously included
Precursor T-cell neoplasms
T-lymphoblastic leukaemia/lymphoma
T-lymphoblastic leukaemia / lymphoma, NOS T-lymphoblastic leukaemia/lymphoma
Early T-precursor lymphoblastic leukaemia / lymphoma Early T-cell precursor lymphoblastic leukaemia
(Entity deleted) NK-lymphoblastic leukaemia/lymphoma
Mature T-cell and NK-cell neoplasms
Mature T-cell and NK-cell leukaemias
T-prolymphocytic leukaemia (Same)
T-large granular lymphocytic leukaemia T-cell large granular lymphocytic leukaemia
NK-large granular lymphocytic leukaemia Chronic lymphoproliferative disorder of NK cells
Adult T-cell leukaemia/lymphoma (Same)
Sezary syndrome (Same)
Aggressive NK-cell leukaemia (Same)
Primary cutaneous T-cell lymphomas
Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder (Same)
Primary cutaneous acral CD8-positive lymphoproliferative disorder Primary cutaneous acral CD8-positive T-cell lymphoma
Mycosis fungoides (Same)
Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Lymphomatoid papulosis (Same)
Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Primary cutaneous anaplastic large cell lymphoma (Same)
Subcutaneous panniculitis-like T-cell lymphoma (Same)
Primary cutaneous gamma/delta T-cell lymphoma (Same)
Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma (Same)
Primary cutaneous peripheral T-cell lymphoma, NOS Not previously included
Intestinal T-cell and NK-cell lymphoid proliferations and lymphomas
Indolent T-cell lymphoma of the gastrointestinal tract Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract
Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract Not previously included
Enteropathy-associated T-cell lymphoma (Same)
Monomorphic epitheliotropic intestinal T-cell lymphoma (Same)
Intestinal T-cell lymphoma, NOS (Same)
Hepatosplenic T-cell lymphoma
Hepatosplenic T-cell lymphoma (Same)
Anaplastic large cell lymphoma
ALK-positive anaplastic large cell lymphoma Anaplastic large cell lymphoma, ALK-positive
ALK-negative anaplastic large cell lymphoma Anaplastic large cell lymphoma, ALK-negative
Breast implant-associated anaplastic large cell lymphoma (Same)
Nodal T-follicular helper (TFH) cell lymphoma
Nodal TFH cell lymphoma, angioimmunoblastic-type Angioimmunoblastic T-cell lymphoma
Nodal TFH cell lymphoma, follicular-type Follicular T-cell lymphoma
Nodal TFH cell lymphoma, NOS Nodal peripheral T-cell lymphoma with TFH phenotype
Other peripheral T-cell lymphomas
Peripheral T-cell lymphoma, not otherwise specified (Same)
EBV-positive NK/T-cell lymphomas
EBV-positive nodal T- and NK-cell lymphoma Not previously included
Extranodal NK/T-cell lymphoma Extranodal NK/T-cell lymphoma, nasal-type
EBV-positive T- and NK-cell lymphoid proliferations and lymphomas of childhood
Severe mosquito bite allergy (Same)
Hydroa vacciniforme lymphoproliferative disorder Hydroa vacciniforme-like lymphoproliferative disorder
Systemic chronic active EBV disease Chronic active EBV infection of T- and NK-cell type, systemic form
Systemic EBV-positive T-cell lymphoma of childhood (Same)