NK/T-cell lymphoma nasal type

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NK/T-cell lymphoma nasal type

A 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.

Clinical findings
Presents as a nasal mass that extends to the skin, as a mid-facial destructive lesion, or as multiple erythaematous nodules which later ulcerate.

Local symptoms
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.

Systemic symptoms
B symptoms (weight loss, fever, fatigue, night sweats, bone pain) occur in less than 20% of patients.

Prognosis
Clinically aggressive; dismal prognosis.
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References in periodicals archive ?
BPDCN had previous names, including blastic NK cell lymphoma and agranular CD4+/CD56+ hematodermic neoplasm, until 2008 at which time the World Health Organization (WHO) renamed this disease BPDCN.
Pathologic examination of the tumor showed NK cell lymphoma.
To the best of our knowledge, however, no true nasal NK cell lymphoma has been described in HIV patients.
NK cell lymphoma is a rare tumor, most common in Asian countries and presenting with distinct clinicopathologic features.
Numerous studies have shown that patients with T and NK cell lymphomas of the sinonasal area have a high incidence of Epstein-Barr virus (EBV) infection.
The oncogenic role of EBV was initially restricted to B-cell lymphomas, but has since been extended to several T-cell and NK cell lymphomas.