Definition

Extranodal NK/T-Cell Lymphoma: A Comprehensive Overview

Definition

Extranodal NK/T-cell lymphoma (ENKTCL) is an aggressive type of non-Hodgkin lymphoma that primarily affects the nasal cavity and other extranodal sites, such as the gastrointestinal tract, skin, and lungs.

Key Features

ENKTCL is characterized by several distinct features:

• Immunophenotypic overlap with systemic EBV-positive T-cell lymphomas
• Aggressive growth pattern and predominantly extranodal involvement
• Angiotropism and angiodestruction, leading to tissue damage and hemorrhage

Origin and Association with Epstein-Barr Virus

ENKTCL is derived from NK cells, which are a type of cytotoxic lymphocyte that forms part of the innate immune system. In most cases, ENKTCL cells are infected with the Epstein-Barr virus (EBV), which is a common herpesvirus that can cause infectious mononucleosis.

The presence of EBV in ENKTCL cells plays a significant role in the development and progression of the disease. EBV provides growth and survival signals to the lymphoma cells, contributing to their aggressive behavior.