Extranodal NK-T-cell lymphoma

Extranodal NK-T-cell lymphoma (ENKTL) is a rare type of non-Hodgkin lymphoma that originates from natural killer cells (NK cells) or T-cells. It is characterized by its extranodal presentation, with the nasal cavity being the most common site of occurrence.

Epidemiology

ENKTL is more prevalent in East Asia and Central and South America, but it can occur in any part of the world. The disease is more common in males than in females, and it typically affects middle-aged adults, although it can occur at any age.

Pathogenesis

The pathogenesis of ENKTL is not fully understood. However, it is known that the disease is closely associated with Epstein-Barr virus (EBV) infection. The virus is thought to infect NK cells or T-cells, leading to their transformation into malignant cells.

Clinical Features

Patients with ENKTL often present with symptoms related to the primary site of disease. For example, patients with nasal ENKTL may experience nasal obstruction, epistaxis, and facial swelling. Systemic symptoms such as fever, weight loss, and night sweats may also occur.

Diagnosis

The diagnosis of ENKTL is based on histopathological examination of the affected tissue. Immunohistochemical staining is used to confirm the presence of NK cells or T-cells. In addition, in situ hybridization for EBV-encoded RNA (EBER) is performed to demonstrate EBV infection.

Treatment

The treatment of ENKTL involves a combination of chemotherapy and radiotherapy. However, the disease is often resistant to conventional chemotherapy regimens, and novel treatment strategies are being explored.

Prognosis

The prognosis of ENKTL is generally poor, particularly for patients with advanced disease. However, early detection and treatment can improve survival rates.

See Also

• Non-Hodgkin lymphoma
• Natural killer cell
• Epstein-Barr virus