Linfoma angiocéntrico centrofacial / Centrofacial angiocentric lymphoma

RESUMEN

El linfoma angiocéntrico centrofacial es una neoplasia linfoide rara, con un diagnóstico a menudo difícil, debido al cuadro clínico inespecífico y a que muchas veces son necesarias varias biopsias para llegar a un diagnóstico correcto. Se trata de un linfoma no Hodgkin(LNH) agresivo, de localización preferente en el tracto respiratorio superior (sobre todo en cavidad nasal), y con un pronóstico ominoso, ya que la supervivencia media es de 12-18 meses aproximadamente(1). Predomina en orientales y sudamericanos, entre los 50-60 añosde edad, y con ligera preferencia por el sexo masculino (21).Se presenta el caso de una paciente ecuatoriana que acude anuestro servicio con edema hemifacial, rinorrea achocolatada y obstrucción respiratoria nasal de 1 mes de evolución, sin respuestaal tratamiento antibiótico y antiinflamatorio, que tras realizarvarias pruebas diagnósticas se evidenció histológicamente lapresencia de un linfoma T extranodal de tipo nasal (también llamado linfoma de células T angiocéntrico)

ABSTRACT

The centrofacial angiocentric lymphoma is a rare lymphoid neoplasm, with an often-difficult diagnosis due to the non-specific clinical picture. On many occasions it is necessary to perform various biopsies to reach the correct diagnosis.This lymphoma is an aggressive Non-Hodgkin`s (NHL) type,which is normally found in the upper respiratory tract (predominantly in the nasal cavity), and has an ominous prognosis, as the average survival rate is between 12 and 18 months (1). It is predominantly found in subjects of oriental and South American extraction, who are between the ages of 50 and 60 years and with a slight tendency towards males (21). This is the case study of a female Ecuadorian patient who was referred to our department with a hemifacial edema, chocolate-like rhinorrhea and nasal respiratory obstruction, which had been treated with antibiotics and anti-inflammatories for a month without success. After performing a number of diagnostic tests, it was found histologically that the patient had an extranodal T-cell lymphoma of the nasal type (also known as T-cell angiocentriclymphoma)