Extranodal nasal NK/T cell lymphoma

Orpha code: 86879OMIM code:

Definition

Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare, malignant neoplasm mainly affecting men in the fifth decade of life, that usually arises in the nose, paranasal sinuses, orbits or upper airway, and that can present with a nasal mass, nasal bleeding, nasal obstruction, palate perforation (i.e. midline perforation of the hard palate), and mid-facial and/or upper airway destructive lesions. In advanced disease stages, which are associated with a poor prognosis, NKTCL may disseminate to other organs. A few cases of NKTCL presenting primarily in the lymph nodes have also been described.

Disease data
Classification

Disease

Synonyms
Angiocentric T-cell lymphoma
Angiocentryczny chłoniak z komórek T
Chłoniak z komórek NK/T
NKTCL
Nosowy chłoniak z komórek T/NK
Ziarniniak pośrodkowy
Lethal midline granuloma
NK/T-cell lymphoma
NKTCL
Nasal T/natural killer-cell lymphoma
ORPHA code
86879
OMIM code
-
ICD10 code
C86.0
ICD11 code
2A90.6

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl