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New approaches to treating primary effusion lymphoma

Introduction: Primary effusion lymphoma (PEL) is rare and aggressive B-cell non-Hodgkin's lymphoma that presents with malignant effusions without tumor masses. Kaposi's sarcoma-associated herpes virus/human herpes virus-8 is universally associated with the pathogenesis of PEL. It is generally resistant to chemotherapy with a short median survival of < 6 months. The optimal treatment for PEL is not well defined and there is a need to establish a standard therapy. Areas covered: This review summarizes the disease features, pathogenesis, diagnosis and current and new targeted treatments for PEL. A literature search on PubMed has been undertaken and the most relevant references have been considered. Expert opinion: Initiating or continuing combination antiretroviral therapy is recommended for HIV-infected PEL patients; however, there is no standard chemotherapy for PEL due to its low incidence and the lack of a large clinical study. PEL is resistant to conventional cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP)-like chemotherapy. Thus, therapeutic approaches that target aggressive and chemotherapy-resistant nature in PEL are considered for treatment. In the use of novel agents, further clinical studies on resistant hematological malignancy, such as multiple myeloma, would provide evidence for their clinical use.