Primary effusion lymphoma

Primary effusion lymphoma (PEL) is a rare subtype of diffuse large B-cell lymphoma (DLBCL). It may also be called body cavity–based lymphoma.

PEL most often occurs in people with weakened immune systems, such as those with HIV or AIDS. It can sometimes occur in people who have had organ transplants. PEL is often linked with Epstein-Barr virus (EBV) infection. It is also linked to Kaposi sarcoma herpes virus (KSHV) infection.

PEL is a very fast-growing (aggressive) type of NHL. It causes an abnormal buildup of fluid in the cavity (space) around the heart, the cavity around the lungs or the cavity in the abdomen. Lymphoma cells are found in the fluid in these body cavities.

Treatments

PEL is treated with chemotherapy. People with HIV or AIDS will also be given highly active antiretroviral therapy (HAART).

Chemotherapy

A combination of chemotherapy drugs is usually used to treat PEL. A common chemotherapy combination used is CHOP:

  • cyclophosphamide (Cytoxan, Procytox)
  • doxorubicin (Adriamycin)
  • vincristine (Oncovin)
  • prednisone

Highly active antiretroviral therapy (HAART)

HAART is a combination of anti-HIV drugs. It is used to treat HIV infection and lower the risk of developing AIDS-related diseases.

Expert review and references

  • American Cancer Society. Non-Hodgkin Lymphoma. 2014: http://www.cancer.org/acs/groups/cid/documents/webcontent/003126-pdf.pdf.
  • American Society of Clinical Oncology . Lymphoma Non-Hodgkin Overview . 2014 : https://www.cancer.net/.
  • Barr PM, Friedberg JW . Rare B cell lymphoproliferative disorders. Raghavan D, Blanke CD, Honson DH, et al (eds.). Textbook of Uncommon Cancer. 4th ed. Wiley Blackwell; 2012: 44: 617 - 626.
  • Medeiros L J . Pathology of non-Hodgkin's and Hodgkin's lymphomas. Wiernik PH, Goldman JM, Dutcher JP, Kyle RA (eds.). Neoplastic Diseases of the Blood. 5th ed. Springer; 2013: 42: 867-918.