Extranodal NK/T-cell lymphoma, nasal type

Definition

An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterised by an often angiocentric and angiodestructive cellular infiltrate composed of Epstein-Barr Virus (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.

Also indexed as

Nearby in Mature T-cell lymphoma, specified types, nodal or systemic

• 2A90.0T-cell prolymphocytic leukaemia
• 2A90.1T-cell large granular lymphocytic leukaemia
• 2A90.2Chronic lymphoproliferative disorders of NK-cells
• 2A90.3Aggressive NK cell leukaemia
• 2A90.4Systemic Epstein-Barr Virus-positive T-cell lymphoma of childhood
• 2A90.5Adult T-cell lymphoma or leukaemia, human T-cell lymphotropic virus type 1-associated
• 2A90.7Enteropathy associated T-cell lymphoma
• 2A90.8Hepatosplenic T-cell lymphoma