Angiocentric lymphoma

Angiocentric lymphoma is a rare type of non-Hodgkin lymphoma that primarily affects the nasal cavity and paranasal sinuses. It is also known as extranodal NK/T-cell lymphoma, nasal type. This disease is characterized by the abnormal proliferation of lymphocytes, a type of white blood cell, in an angiocentric pattern (surrounding and invading blood vessels).

Epidemiology

Angiocentric lymphoma is more common in East Asia and Central and South America than in North America and Europe. It affects more males than females and is most commonly diagnosed in middle-aged adults, although it can occur at any age.

Pathogenesis

The pathogenesis of angiocentric lymphoma is not fully understood. It is associated with Epstein-Barr virus (EBV) infection, suggesting that EBV may play a role in the development of this disease. The lymphoma cells in angiocentric lymphoma are usually of the T-cell or natural killer (NK) cell type, and they often express CD56, a marker of NK cells.

Clinical Features

Patients with angiocentric lymphoma typically present with nasal obstruction, recurrent epistaxis (nosebleeds), and facial swelling. Other symptoms may include fever, weight loss, and night sweats. The disease often invades adjacent structures, leading to eye symptoms, skin lesions, and neurological symptoms.

Diagnosis

The diagnosis of angiocentric lymphoma is based on histopathological examination of a biopsy specimen. The lymphoma cells show an angiocentric and angiodestructive growth pattern, leading to necrosis of the affected tissue. Immunohistochemical staining is used to determine the cell type and the presence of EBV.

Treatment

The treatment of angiocentric lymphoma includes chemotherapy, radiation therapy, and in some cases, stem cell transplantation. The choice of treatment depends on the stage of the disease, the patient's overall health, and other factors.

Prognosis

The prognosis of angiocentric lymphoma is generally poor, with a 5-year survival rate of less than 50%. Factors that are associated with a worse prognosis include advanced stage of the disease, high International Prognostic Index score, and presence of systemic symptoms.

See Also

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