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 ?
The subtype distribution of PTCL is also significantly different between China and Western countries, in which extranodal NK/T-cell lymphoma, nasal type (ENKL), a subtype highly aggressive with very poor prognosis, to be the leading subtype in Chinese population.
Based on the histopathologic and immunophenotyping results, a final diagnosis of extranodal NK/T-cell lymphoma, nasal type, was made.
Since the nasopharynx and the nasal cavity are the most common sites of the NK/T-cell type of lymphoma, it is also called the nasal type.
69,70) T-cell lymphomas can involve any part of the gastrointestinal tract especially in patients in the Far East, and whereas some cases may be examples of EATL, the Far East is nonendemic for celiac disease and most cases in this geographic region are peripheral T-cell lymphomas, not otherwise specified (NOS), NK/T-cell lymphomas of nasal type, and cases linked with the human T-cell lymphotropic virus type 1.
69,72,74,77) For example, the extranodal NK/T-cell lymphomas of nasal type that present in intestine exhibit the usual broad cytologic spectrum of that form of lymphoma, ranging from small to intermediate-size to large cells with marked nuclear pleomorphism.
Similar to true NK-cell lymphomas of extranodal nasal type, the T-cell receptor in the enteropathy cases is germline; but, in contrast to NK nasal-type lymphomas, studies for Epstein-Barr virus using Epstein-Barr virus-encoded RNA yield negative results.
It was also CD57 negative and consistent with the diagnosis of an extranodal NK/T-cell lymphoma, nasal type.
Ki-67 expression is predictive of prognosis in patients with stage I/II extranodal NK/T-cell lymphoma, nasal type.
6-8) Epstein-Barr virus-associated neoplasms, such as nasopharyngeal carcinoma and extranodal natural killer (NK)/T-cell lymphoma, nasal type (NKTCL), are prevalent in Taiwan.
EBER ISH and Extranodal NK/T-cell Lymphoma, Nasal Type
Natural killer/T-cell lymphoma of nasal type primarily affects men and is a disease of adults, with a median age in the sixth decade of life.
Natural killer/T-cell lymphoma of nasal type commonly presents as a destructive process of the midfacial region, with nasal septal destruction, palatal destruction/perforation, or orbital swelling, or with obstructive symptoms related to a mass.