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Non-Hodgkin lymphoma

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Primary effusion lymphoma (PEL)

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:

  • pleural space (the space between the 2 layers of pleurapleuraThe thin layer of tissue that covers the lungs and lines the chest cavity. It protects and cushions the lungs and produces a fluid that acts like a lubricant so the lungs can move smoothly in the chest cavity.)
  • peritoneal space in the abdominal cavity
  • pericardial space (the space between the layers of tissue that surround the heart)

Lymphoma cells are found in the fluid in these body cavities.

People with PEL often have a very poor prognosis.

Treatment

Treatment of PEL is usually chemotherapy.

Chemotherapy

A combination of chemotherapy drugs is usually used to treat PEL. The same combination used to treat DLBCL is often used.

  • CHOP – cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone (Deltasone)

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.

Antiretroviral therapy

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.

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