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Primary effusion lymphoma (PEL) is rare subtype of diffuse large B-cell lymphoma (DLBCL). It is also considered a distinct type of non-Hodgkin lymphoma (NHL) in the World Health Organization (WHO) classification system. PEL may also be called body cavity lymphoma.
PEL is linked to human herpes virus 8 (HHV8) infection. It is also associated with Epstein-Barr virus (EBV) infection in many cases. PEL most often occurs in people with weakened immune systems, such as those with HIV/AIDS. It can sometimes occur in people who have had organ transplants.
PEL is a very fast-growing (aggressive) lymphoma. There is often an abnormal buildup of fluid in body cavities, usually in:
Lymphoma cells are found in the fluid in these body cavities.
People with PEL often have a very poor prognosis.
Treatment of PEL is usually chemotherapy.
A combination of chemotherapy drugs is usually used to treat PEL. The same combination used to treat DLBCL is often used.
Rituximab (Rituxan) is a type of biological therapy that is often used for many B-cell types of NHL, but it does not play a role in treating PEL. This is because PEL cells do not usually express CD20, a protein found on several cancerous B cells. Rituximab targets this protein, so it would not be effective in treating PEL.
Highly active antiretroviral therapy (HAART) is a combination of anti-HIV drugs. It is used for treating HIV infection and to reduce the risk of developing AIDS-related diseases. HAART is used in addition to chemotherapy if the person with PEL is HIV positive.
For more detailed information on specific drugs, go to sources of drug information.