Primary effusion lymphoma

Primary Effusion Lymphoma

Micrograph of Primary Effusion Lymphoma

Primary Effusion Lymphoma (PEL) is a rare type of non-Hodgkin lymphoma that is characterized by the presence of malignant lymphocytes in the body cavities without the formation of a solid tumor mass. It is most commonly associated with human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV).

Pathophysiology

PEL is primarily linked to infection with HHV-8, which is found in the tumor cells of nearly all cases. The virus is thought to play a crucial role in the transformation of normal lymphocytes into malignant cells. In addition to HHV-8, many patients with PEL are also co-infected with human immunodeficiency virus (HIV), which contributes to the immunocompromised state that allows for the development of this lymphoma.

Clinical Presentation

Patients with PEL typically present with symptoms related to the accumulation of fluid in the body cavities, such as the pleural cavity, peritoneal cavity, or pericardial cavity. Common symptoms include dyspnea, abdominal distension, and chest pain. Unlike other lymphomas, PEL does not usually form a solid tumor mass, and the diagnosis is often made by analyzing the fluid from the affected cavity.

Diagnosis

The diagnosis of PEL is made by cytological examination of the effusion fluid, which reveals large, atypical lymphoid cells. Immunophenotyping and molecular studies are used to confirm the presence of HHV-8 in the tumor cells. The cells typically express markers such as CD45 and CD30, but lack expression of B-cell markers like CD20.

Treatment

The treatment of PEL is challenging due to the aggressive nature of the disease and the frequent presence of co-morbid conditions such as HIV infection. Chemotherapy regimens similar to those used for other aggressive lymphomas, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), are commonly employed. Antiretroviral therapy is also crucial for patients with HIV to improve immune function and control viral replication.

Prognosis

The prognosis for patients with PEL is generally poor, with a median survival of less than one year. Factors that influence prognosis include the patient's overall health, the presence of HIV infection, and the response to treatment.

Related Pages

• Non-Hodgkin lymphoma
• Human herpesvirus 8
• Kaposi's sarcoma
• Human immunodeficiency virus

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