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 ?
Yamaguchi et al., "Prognostic factors for mature natural killer (NK) cell neoplasms: aggressive NK cell leukemia and extranodal NK cell lymphoma, nasal type," Annals of Oncology, vol.
6, dead MTx + Rd 49, F [2] MTx 48, alive 26, F [17] -- 0.5, dead Hypo: anterior hypopituitarism; B-cell: B-cell lymphoma; Chemo: chemotherapy; CN: cranial nerve palsy; DI: diabetes insipidus; F, S&WL: fever, night sweat, and weight loss; HA: headache; HD: high dose; i.t.: intrathecal; MTx: methotrexate; n.d.: not described; NK: NK cell lymphoma; Nys: nystagmus; OS: overall survival; PRL: hyperprolactinemia; Rd: radiotherapy; Ste: steroid; T-cell: T-cell lymphoma;--: none.
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.
Approximately 80% of the reported NL cases originate from B-cells.[sup][4] However, NK cell lymphoma is very rare, and the present case was finally diagnosed as ENKL pathologically.
To the best of our knowledge, however, no true nasal NK cell lymphoma has been described in HIV patients.
This first report of nasal NK cell lymphoma occurring in an HIV patient shows that EBV involvement in HIV-associated non-B-cell lymphomas may represent a further manifestation of opportunistic EBV infection arising in these immunocompromised hosts.
Thus, a combination of immunophenotypic studies and molecular biological studies is essential for the differential diagnosis of T-cell and NK cell lymphomas. The characteristics of the major differential diagnoses are listed in Table 1.
Freud et al., "The Epstein-Barr virus (EBV) in T cell and NK cell lymphomas: time for a reassessment," Current Hematologic Malignancy Reports, vol.
Freud et al., "The epsteinbarr virus (EBV) in T cell and NK cell lymphomas: time for a reassessment," Current Hematologic Malignancy Reports, vol.
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.[10] It is noteworthy that HHV-8 sequences have been detected in a few cases of angioimmunoblastic lymphadenopathy, a disease now considered a T-cell lymphoma.[9]