Cutaneous T-Cell Lymphoma and NK Cells

  • Lisia NudelmannEmail author
  • Renan Rangel Bonamigo


Primary cutaneous lymphomas (PCLs) are a group of extranodal non-Hodgkin lymphomas characterized by early accumulation of mononuclear cells, most of them lymphocytes, on skin, with no evidence of extracutaneous disease at diagnosis. T-cell and natural killer (NK) cell lymphomas (CTCL/NK) with an absolute predominance of mycosis fungoides (MF) represent approximately 44% of all PCLs. The etiology and the exact steps in the pathogenesis of CTCL are not understood. It is more reasonable to assume that these diseases represent the endpoint of several different processes. Genetic, environmental, and infectious factors should all be considered in the etiology of CTCL. The current classification for PCLs was proposed in 2005 by the World Health Organization (WHO) and the European Organization for Research and Treatment of Cancer (EORTC). WHO-EORTC Classification for primary cutaneous T- and NK-cell lymphomas is MF and subtypes of MF (folliculotropic MF, pagetoid reticulosis, granulomatous cutis laxa); Sézary syndrome; adult T-cell leukemia/lymphoma; primary cutaneous CD30+ lymphoproliferative diseases (primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis); subcutaneous panniculitis-like T-cell lymphoma; extranodal NK/T-cell lymphoma, nasal type; and primary cutaneous peripheral T-cell lymphoma, unspecified (primary aggressive epidermotropic CD8+ cutaneous T-cell lymphoma, provisional entity; cutaneous γδ T-cell lymphoma, provisional entity; and cutaneous lymphoma of small and medium pleomorphic CD4+ T-cell, provisional entity).


Cutaneous T-cell lymphoma Non-Hodgkin lymphoma Mycosis fungoides Sézary syndrome Adult T-cell leukemia/lymphoma Primary cutaneous CD30+ lymphoproliferative diseases Primary cutaneous anaplastic large cell lymphoma Lymphomatoid papulosis Subcutaneous panniculitis-like T-cell lymphoma Extranodal NK/T-cell lymphoma, nasal type Primary cutaneous peripheral T-cell lymphoma 




Arrangement of cells around the vessels; perivascular.


Movement toward the epidermis, as in the migration of T lymphocytes into the epidermis in mycosis fungoides.


A process of detecting antigens (e.g., proteins) in cells of a tissue section by exploiting the principle of antibodies binding specifically to antigens in biological tissues


A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells with normal cells of the immune system.

Pautrier’s microabscess

A well-defined collection of cells within the epidermis in T-cell lymphoma and mycosis fungoides.

Reed–Stenberg cell

A giant binucleated or multinucleated macrophage, characteristic of Hodgkin’s disease.

Sézary cell

An atypical mononuclear cell seen in the peripheral blood in Sézary syndrome; has a large, convoluted nucleus and scant cytoplasm containing vacuoles that test positive with the periodic acid-Schiff stain.


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Copyright information

© Springer International Publishing Switzerland 2018

Authors and Affiliations

  1. 1.University Hospital, Luteran University of Brazil, ULBRACanoasBrazil
  2. 2.Dermatology Service, Hospital de Clínicas de Porto Alegre (HCPA), Federal University of Rio Grande do Sul (UFRGS)Porto AlegreBrazil
  3. 3.Sanitary Dermatology Service of the Department of Health of Rio Grande do Sul State (ADS-SES)Porto AlegreBrazil
  4. 4.Graduate Pathology Program of Federal University of Health Sciences of Porto AlegrePorto AlegreBrazil

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