ICD-11 classes
02 Neoplasms
Neoplasms of haematopoietic or lymphoid tissues
Mature T-cell or NK-cell neoplasms
2A90 — Mature T-cell lymphoma, specified types, nodal or systemic
2A90.6 — Extranodal NK/T-cell lymphoma, nasal type
ICD-11 2A90.6 — Extranodal NK/T-cell lymphoma, nasal type
An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterised 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.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: