Primary effusion lymphoma in the absence of HIV infection--clinical presentation and management

Primary Effusion Lymphoma (PEL) is a rare type of Non-Hodgkin's Lymphoma caused by human herpesvirus type 8, also termed Kaposi's sarcoma-associated herpesvirus. It usually occurs in human immunodeficiency virus (HIV)-infected patients. A subset of patients is not infected with HIV and their treatment remains poorly defined. To clarify treatment issues in HIV-negative PEL patients, we report on two such patients who represent two opposing ends in the spectrum of treatment and review the literature regarding treatment options and patient outcomes. Either repeated cycles of chemotherapy or, surprisingly, drainage of the malignant effusions alone, proved very effective in our patients. The literature reveals additional treatment options which may be effective including immunochemotherapy, stem cell transplantation, antiviral treatment and immunomodulatory and targeted biological therapy. However, no controlled trials were found due to the rarity of the condition. In the absence of controlled trials, treatment decisions in PEL not associated with HIV must remain individual and patient-tailored.