ICD-11 code : 2A90.6;
Preferred Label : Extranodal NK/T-cell lymphoma, nasal type;
ICD-11 definition : An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is
characterized by an often angiocentric and angiodestructive cellular infiltrate composed
of EBV positive NK/T cells. The nasal cavity is the most common site of involvement.
Patients often present with midfacial destructive lesions (lethal midline granuloma).
The disease may disseminate rapidly to various anatomic sites including the gastrointestinal
tract, skin, testis, and cervical lymph nodes. It is also known as angiocentric T-cell
lymphoma. The term polymorphic reticulosis has been widely used to describe the morphologic
changes seen in this type of lymphoma. However, the latter term may also apply to
lymphomatoid granulomatosis, which is an angiocentric and angiodestructive EBV positive
B-cell lymphoproliferative disorder.;
Origin ID : 684005900;
UMLS CUI : C0392788;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- ICD-10 Mapping
- Semantic type(s)
- UMLS correspondences (same concept)
- Validated automatic mappings to BTNT
An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is
characterized by an often angiocentric and angiodestructive cellular infiltrate composed
of EBV positive NK/T cells. The nasal cavity is the most common site of involvement.
Patients often present with midfacial destructive lesions (lethal midline granuloma).
The disease may disseminate rapidly to various anatomic sites including the gastrointestinal
tract, skin, testis, and cervical lymph nodes. It is also known as angiocentric T-cell
lymphoma. The term polymorphic reticulosis has been widely used to describe the morphologic
changes seen in this type of lymphoma. However, the latter term may also apply to
lymphomatoid granulomatosis, which is an angiocentric and angiodestructive EBV positive
B-cell lymphoproliferative disorder.
