Cutaneous T-Cell Lymphoma and NK Cells
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).
KeywordsCutaneous 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.
A well-defined collection of cells within the epidermis in T-cell lymphoma and mycosis fungoides.
A giant binucleated or multinucleated macrophage, characteristic of Hodgkin’s disease.
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.
- 11.Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. J Am Acad Dermatol. 2014;70(2):205.e1–16. quiz 221–2CrossRefGoogle Scholar
- 42.Paulli M, Berti E. Cutaneous T-cell lymphomas (including rare subtypes). Curr Concepts II Haematol. 2004;89(11):1372–88.Google Scholar
- 43.Kim YH, Willemze R, Pimpinelli N, Whittaker S, Olsen EA, Ranki A, et al. TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110(2):479–84.CrossRefPubMedGoogle Scholar
- 44.Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, et al. Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110(6):1713–22.CrossRefPubMedGoogle Scholar
- 45.Rosen ST, Querfeld C. Primary cutaneous T-cell lymphomas. Hematol Am Soc Hematol Educ Program. 2006;323–30:513.Google Scholar
- 46.Weberschock T, Strametz R, Lorenz M, Röllig C, Bunch C, Bauer A, et al. Interventions for mycosis fungoides. Cochrane Database Syst Rev. 2012;9:CD008946.Google Scholar
- 51.Whittaker SJ, Marsden JR, Spittle M, Russell Jones R, British Association of Dermatologists, U.K. Cutaneous Lymphoma Group. Joint British Association of Dermatologists and U.K. Cutaneous Lymphoma Group guidelines for the management of primary cutaneous T-cell lymphomas. Br J Dermatol. 2003;149(6):1095–107.CrossRefPubMedGoogle Scholar
- 53.Hermine O, Allard I, Lévy V, Arnulf B, Gessain A, Bazarbachi A, et al. A prospective phase II clinical trial with the use of zidovudine and interferon-alpha in the acute and lymphoma forms of adult T-cell leukemia/lymphoma. Hematol J Off J Eur Haematol Assoc EHA. 2002;3(6):276–82.Google Scholar
- 57.Bekkenk MW, Geelen FA, van Voorst Vader PC, Heule F, Geerts ML, van Vloten WA, et al. Primary and secondary cutaneous CD30(+) lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood. 2000;95(12):3653–61.PubMedGoogle Scholar