Primary effusion lymphoma: current concepts and management

To summarize the current epidemiology, management, and outcomes of primary effusion lymphoma (PEL) and highlight possible future research efforts.

Cyclophosphamide, doxorubicin, vincristine, prednisone-based chemotherapy regimens alone or in combination with immunomodulatory agents (e.g., lenalidomide), or proteasome inhibitors (e.g., bortezomib), or targeted therapies, are commonly used to treat PEL. Highly active antiretroviral therapy should be continued or initiated in patients with HIV infection. Randomized controlled trials are lacking. Prognosis remains grim and there exists a need for further investigation into optimal treatment strategies.

PEL is an aggressive mature B-cell neoplasm primarily seen in young to middle aged men with HIV, though immunosuppression related to age and comorbidities such as cirrhosis or organ transplantation also predisposes to PEL. Classic cavitary PEL presents as an effusion in the pleural, pericardial, or peritoneal space. Human herpes virus-8/Kaposi's sarcoma herpes virus) is classically detected. Given its rarity, randomized controlled trials evaluating optimal treatment regimens are lacking, and cyclophosphamide, doxorubicin, vincristine, prednisone-based chemotherapy has been the mainstay of treatment. Advancement in knowledge of the oncogenic signaling pathways involved in Kaposi's sarcoma herpes virus-induced tumorigenesis may pave the way to develop targeted therapies. VIDEO ABSTRACT.