Linfoma nasal de células T/NK

Abstract

Nasal T-cell and Natural Killer cell lymphoma (NT/NKL), having been given many names, was defined and described in the year 2001 by the World Health Organization (WHO), on the basis of a previous classification by the Revised European-American Lymphoma Classification (REAL) as it is known today. Its incidence in the western world is low, while in Asia it represents the second most frequent group of lymphomas, followed by the gastrointestinal [lymphoma]. It is typically located in the nasal cavity and maxillary sinuses. It is associated with an aggressive clinical course, characterized by the destruction of surrounding tissue. The definitive diagnosis is made by means of in situ hybridization techniques, in order to determine the immunophenotype. Its association with the Epstein-Barr virus (EBV) has frequently been observed. The prognosis of this disease is determined by the International Prognosis Index (IPI) and by the size of the tumor. In spite of being responsive to irradiation therapy, its prognosis is gloomy, and the death of the patient occurs shortly after the diagnosis, generally as a result of treatment complications.