Intractable Rare Dis Res. 2014;3(3):65-74. (DOI: 10.5582/irdr.2014.01010)

Current status of treatment for primary effusion lymphoma.

Okada S, Goto H, Yotsumoto M

Primary effusion lymphoma (PEL) is a rare and aggressive B-cell non-Hodgkin's lymphoma that usually presents with malignant effusions without tumor masses. An extracavitary or solid variant of PEL has also been described. Human herpes virus 8/Kaposi sarcomaassociated herpes virus (HHV-8/KSHV) is universally associated with the pathogenesis of PEL. More than 70% of cases occur with concurrent Epstein-Barr virus infection, but its relation to the pathogenesis is unknown. Patients are found in the context of immunosuppressive states (HIV-1 infection, post-organ transplantation). PEL is usually treated with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)-like chemotherapy with antiretroviral therapy if HIV-1 is positive. However, it is generally resistant to chemotherapy with a short median survival of less than 6 months. The optimal treatment for PEL has not been established yet. More intensive chemotherapy, such as doseadjusted EPOCH (DA-EPOCH; etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin) and CDE (cyclophosphamide, doxorubicin, etoposide) are expected to show a favorable prognosis. Recently, the molecular steps in KSHV/HHV-8-driven oncogenesis have begun to be revealed, and molecular targeting therapies such as proteasome, NF-κB, cytokines and surface antigens would provide evidence for their clinical use.

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