| dbo:abstract |
Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) (also termed angiocentric lymphoma, nasal-type NK lymphoma, NK/T-cell lymphoma, polymorphic/malignant midline reticulosis, and lethal midline granuloma) is a rare type of lymphoma that commonly involves midline areas of the nasal cavity, oral cavity, and/or pharynx At these sites, the disease often takes the form of massive, necrotic, and extremely disfiguring lesions. However, ENKTCL-NT can also involve the eye, larynx, lung, gastrointestinal tract, skin, and various other tissues. ENKTCL-NT mainly affects adults; it is relatively common in Asia and to lesser extents Mexico, Central America, and South America but is rare in Europe and North America. In Korea, ENKTCL-NT often involves the skin and is reported to be the most common form of cutaneous lymphoma after mycosis fungoides. ENKTCL-NT is classified as an Epstein-Barr virus-associated lymphoproliferative disease. It is due to the malignant transformation of either one of two types of lymphocytes, NK cells or a T cell variant termed cytotoxic T cells, that are infected with the Epstein–Barr virus (EBV). Typically, the viral infection, which affects >90% of the world population, occurs years before evidence of ENKTCL-NT, is carried in cells in a latent, asymptomatic form, and for unclear reasons becomes active in causing the disease. Following the virus's activation, the infected cells acquire numerous genetic abnormalities which may play an important role in the development and/or progression of ENKTCL-NT. Epstein-Barr virus-positive nodal NK/T cell lymphoma (EBV+ nodal NKTCL) was considered to be one form of ENKTCL-NT since it is a malignancy of EBV-infected NK or T cells. However, EBV+ nodal NKTCL is manifested primarily by its involvement in lymph nodes; it also has clinical, pathological, pathophysiological, and genetic features that differ significantly from those of ENKTCL-NT. The World Health Organization, 2016, therefore reclassified this lymphoma as a variant of a disease to which its features more closely resemble, peripheral T-cell lymphoma not otherwise specified. While a rare disease, particularly in North America, ENKTCL-NT has recently gained much interest. Clinical studies have found that newer chemotherapeutic regimens greatly improved survival in cases of early disease. While, survival in advanced cases is still extremely poor, generally being only a few months, recent studies suggest that new regimens directed at gene mutation and expression abnormalities may improve survival. Further study of these new regimens has important implications not only for ENKTCL-NT but also for other NK/T cell malignancies. (en) |
| rdfs:comment |
Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) (also termed angiocentric lymphoma, nasal-type NK lymphoma, NK/T-cell lymphoma, polymorphic/malignant midline reticulosis, and lethal midline granuloma) is a rare type of lymphoma that commonly involves midline areas of the nasal cavity, oral cavity, and/or pharynx At these sites, the disease often takes the form of massive, necrotic, and extremely disfiguring lesions. However, ENKTCL-NT can also involve the eye, larynx, lung, gastrointestinal tract, skin, and various other tissues. ENKTCL-NT mainly affects adults; it is relatively common in Asia and to lesser extents Mexico, Central America, and South America but is rare in Europe and North America. In Korea, ENKTCL-NT often involves the skin and is reported to be the most common form (en) |
