Primary effusion lymphoma

Primary effusion lymphoma (PEL) is a rare type of non-Hodgkin lymphoma that is often associated with human herpesvirus 8 (HHV8) infection. It is also known as body cavity-based lymphoma and is characterized by the presence of lymphomatous effusions in body cavities without detectable tumor masses.

Etiology

PEL is strongly associated with HHV8 infection, also known as Kaposi's sarcoma-associated herpesvirus (KSHV). The virus is found in all PEL cells and is thought to play a key role in the development of the disease. PEL is also frequently associated with co-infection with Epstein-Barr virus (EBV).

Clinical Features

PEL typically presents as a lymphomatous effusion in the pleural, pericardial, or peritoneal cavities. It is often associated with immunodeficiency, particularly in individuals with HIV/AIDS. Symptoms can include fever, night sweats, weight loss, and dyspnea.

Diagnosis

Diagnosis of PEL is based on cytological examination of the effusion fluid, which typically shows large, atypical lymphoid cells. Immunophenotyping can help to confirm the diagnosis, with PEL cells typically expressing CD45, CD30, and CD38, but lacking B-cell markers.

Treatment

Treatment of PEL is challenging due to its aggressive nature and association with immunodeficiency. Antiviral therapy against HHV8 and EBV may be used, along with chemotherapy and immunotherapy.

Prognosis

The prognosis of PEL is generally poor, with a median survival time of less than 6 months. However, survival can be improved with early diagnosis and treatment.

See Also

• Non-Hodgkin lymphoma
• Human herpesvirus 8
• Epstein-Barr virus
• HIV/AIDS

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