Table 1

Old and new WHO classifications of PTCLs

Old WHO classificationNew WHO classification
Precursor T-cell lymphoma  
T-lymphoblastic lymphoma/leukemia  
Mature T-cell lymphomas  
T-cell prolymphocytic leukemia T-cell prolymphocytic leukemia 
T-cell granular lymphocytic leukemia T-cell large granular lymphocytic leukemia 
Aggressive NK-cell leukemia Aggressive NK-cell leukemia 
 Indolent large granular NK-cell lymphoproliferative disorder (provisional) 
ATL/adult T-cell leukemia (HTLV1+ATL/adult T-cell leukemia 
Extranodal NK-/T-cell lymphoma, nasal type Extranodal NK-/T-cell lymphoma, nasal type 
Enteropathy-type T-cell lymphoma EATL 
Hepatosplenanic T-cell lymphoma Hepatosplenic T-cell lymphoma 
Subcutaneous panniculitis-like T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma (αβ only) 
 Primary cutaneous γδ T-cell lymphoma 
Mycosis fungoides/Sézary syndrome Mycosis fungoides/Sézary syndrome 
Anaplastic large-cell lymphoma, systemic or cutaneous ALCL: ALK+ 
 ALCL: ALK (provisional) 
PTCL, unspecified PTCL, NOS 
AITL AITL 
 Primary cutaneous CD30+ T-cell LPD 
     LYP and primary cutaneous ALCL 
 Primary cutaneous CD4+ small/medium T-cell lymphoma (provisional) 
 Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (provisional) 
 Systemic EBV+ T-cell LPD of childhood 
 Hydroa vacciniforme-like lymphoma 
Old WHO classificationNew WHO classification
Precursor T-cell lymphoma  
T-lymphoblastic lymphoma/leukemia  
Mature T-cell lymphomas  
T-cell prolymphocytic leukemia T-cell prolymphocytic leukemia 
T-cell granular lymphocytic leukemia T-cell large granular lymphocytic leukemia 
Aggressive NK-cell leukemia Aggressive NK-cell leukemia 
 Indolent large granular NK-cell lymphoproliferative disorder (provisional) 
ATL/adult T-cell leukemia (HTLV1+ATL/adult T-cell leukemia 
Extranodal NK-/T-cell lymphoma, nasal type Extranodal NK-/T-cell lymphoma, nasal type 
Enteropathy-type T-cell lymphoma EATL 
Hepatosplenanic T-cell lymphoma Hepatosplenic T-cell lymphoma 
Subcutaneous panniculitis-like T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma (αβ only) 
 Primary cutaneous γδ T-cell lymphoma 
Mycosis fungoides/Sézary syndrome Mycosis fungoides/Sézary syndrome 
Anaplastic large-cell lymphoma, systemic or cutaneous ALCL: ALK+ 
 ALCL: ALK (provisional) 
PTCL, unspecified PTCL, NOS 
AITL AITL 
 Primary cutaneous CD30+ T-cell LPD 
     LYP and primary cutaneous ALCL 
 Primary cutaneous CD4+ small/medium T-cell lymphoma (provisional) 
 Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (provisional) 
 Systemic EBV+ T-cell LPD of childhood 
 Hydroa vacciniforme-like lymphoma 

The WHO classification for PTCLs was updated in 2008. The new classification expanded some existing disease types and added several new provisional diseases.

WHO indicates World Health Organization; PTCL, peripheral T-cell lymphoma; NK, natural killer; HTLV1, human T-lymphotropic virus type 1; EATL, enteropathy-associated T-cell lymphoma; and ALCL, anaplastic large cell lymphoma; AITL, angioimmunoblastic T-cell lymphoma; and NOS, not otherwise specified.

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