Combined chemotherapy including high‐dose methotrexate in KSHV/HHV8‐associated primary effusion lymphoma

Primary effusion lymphoma (PEL) is a rare KSHV/HHV8‐associated high‐grade non‐Hodgkin's lymphoma (NHL) of B‐cell origin, characterized by serous effusions in body cavities. Most patients are HIV‐infected homosexual men with severe immunosuppression and other KSHV/HHV8‐associated diseases such as Kaposi's sarcoma (KS). The prognosis is poor with a median survival of less than 6 months in most cohorts. The achievement of a sustained complete remission is rare. High‐dose chemotherapy regimens are warranted to improve complete remission rate and survival. Seven patients with AIDS‐associated PEL were treated with a combined chemotherapy including high‐dose methotrexate followed by leucovorin rescue. In all cases, KSHV/HHV8 sequences were detected in the effusion samples using quantitative PCR assays. Five patients had a pre‐existing KS, associated in three cases with multicentric Castleman's disease (MCD). Upon diagnosis, 6 patients received antiretroviral therapy, which was maintained during chemotherapy in 5 of them. At time of analysis, 3 out of 7 patients were in complete remission 18, 26, and 78 months after PEL diagnosis. Three patients died with a progressive PEL at 22, 67, and 153 days after diagnosis, and 1 patient died 9 months after PEL diagnosis with a MCD‐associated plasmablastic NHL. Complete remission was obtained in 3 out of 7 patients treated for AIDS‐associated PEL with combined chemotherapy containing high‐dose methotrexate. Am. J. Hematol. 73:143–148, 2003. © 2003 Wiley‐Liss, Inc.