title: Current status of treatment for primary effusion lymphoma.
abstract: 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 sarcoma-associated 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 dose-adjusted 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.
authors.affiliation: Division of Hematopoiesis, Center for AIDS Research, Kumamoto University, Kumamoto, Japan;
authors.forename: Seiji
authors.initials: S
authors.lastname: Okada
authors.affiliation: Division of Hematopoiesis, Center for AIDS Research, Kumamoto University, Kumamoto, Japan;
authors.forename: Hiroki
authors.initials: H
authors.lastname: Goto
authors.affiliation: Department of Laboratory Medicine, Tokyo Medical University, Tokyo, Japan.
authors.forename: Mihoko
authors.initials: M
authors.lastname: Yotsumoto
keywords: HIV-1/AIDS; Human herpes virus-8/Kaposi sarcoma-associated herpes virus (HHV-8/KSHV); NF-κB; PEL xenograft mouse model; Primary effusion lymphoma (PEL); combination antiretroviral therapy (cART)