NK/T-cell lymphoma nasal type
NK/T-cell lymphoma nasal typeA primary lymphoma of the nasal cavity which is linked to Epstein-Barr virus infection, HIV, inherited immunodeficiencies (e.g., ataxia-telangiectasia), common-variable immunodeficiency disease, Chédiak-Higashi syndrome, autoimmune disease (e.g., Sjögren syndrome), coeliac disease, and chemical exposures (e.g., dioxin, herbicides, phenytoin, prior radiation or chemotherapy). It is remarkable for having a much higher mortality and much lower response to chemo- and radiotherapy.
Presents as a nasal mass that extends to the skin, as a mid-facial destructive lesion, or as multiple erythaematous nodules which later ulcerate.
Facial pain and swelling, diplopia, proptosis, loss of visual acuity, orbital mass and swelling, nasal obstruction, septal perforation, purulent discharge, sinusitis, epistaxis, hearing loss, oral ulcers, odynophagia, dysphagia, velopharyngeal incompetence, trismus, halitosis, otalgia, neck mass.
B symptoms (weight loss, fever, fatigue, night sweats, bone pain) occur in less than 20% of patients.
Clinically aggressive; dismal prognosis.