Linfoma de células T/Natural Killer extranodal, tipo nasal

Abstract

Introducción: El linfoma de células T/Natural Killer (T/NK) extranodal, tipo nasal, es un linfoma extra-ganglionar poco frecuente, con extensión a lo largo de la línea media facial, rápidamente progresivo, catastrófico y de mal pronóstico, por lo que también se le conoce como “granuloma letal de la línea media”. Objetivo: El propósito de este artículo es revisar la literatura disponible y actualizada sobre el linfoma de células T/NK extranodal, tipo nasal: manifestaciones clínicas, estándares de enfoque, diagnóstico, pronóstico y tratamiento. Diseño: Revisión Narrativa de la literatura. Metodología: Revisión de la literatura mediante búsqueda selectiva por términos MeSH: Extranodal Natural Killer /T cell lymphoma, nasal type, lethal midline granuloma, de las bases de datos: MEDLINE, Current Contents, Cochrane, Pubmed y Scielo, entre los años 2000 y 2014. Resultados: Se revisaron 36 artículos según los requerimientos de los objetivos. El linfoma de células T/NK es una neoplasia rápidamente progresiva, destructiva y de mal pronóstico excepto en los casos donde el diagnóstico ha sido oportuno. Conclusiones: El diagnóstico y tratamiento tempranos del linfoma de células T/NK extranodal, tipo nasal, son las únicas herramientas para mejorar el mal pronóstico y gran afectación en la calidad de vida de los pacientes con esta enfermedad.

Linfoma extranodal nasal de células T/NK. Reporte de Caso

“…T/NK cell lymphomas, formerly known as lethal midline granulomas, are neoplasms derived from mature T and NK cells; of these, ENKL is more common in Asian and Latin American populations (3).…”

“…Since the clinical course of ENKL varies depending on the stage of the disease, it is common that differential diagnoses, such as acute or chronic sinusitis, are considered early in the disease, as well as infectious processes of fungal etiology (fungal rhinosinusitis) with multiple treatments without improvement. Regarding disease progression, other differential diagnoses to consider in the presence of B symptoms include squamous cell carcinoma and nasopharyngeal non-keratinizing carcinoma, which are clinically similar to ENKL (only in immunohistochemistry), lymphomatoid granulomatosis, which is caused by B cells, and diffuse B-cell lymphoma in elderly patients, which has no regular involvement in the sinus or nasal areacv (3,5,12).…”

“…Mexico, Guatemala, and Peru are the countries with the most reports of this disease in Latin America. However, it should be noted that there are still no statistical data to establish a relationship between the geographical location of patients and the development of this type of tumor (1,3).…”

“…Although the etiology of this disease is unknown, some authors consider Epstein-Barr virus as an oncogenic factor because this infection causes neoplastic transformation of natural killer (NK) cells. Moreover, this herpesvirus has been found in almost all patients with ENKL and persists in studies in people with poor response to treatment (3)(4)(5).…”