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

✍ Scribed by Emmanuelle Boulanger; Marie-Thérèse Daniel; Félix Agbalika; Eric Oksenhendler


Publisher
John Wiley and Sons
Year
2003
Tongue
English
Weight
120 KB
Volume
73
Category
Article
ISSN
0361-8609

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✦ Synopsis


Abstract

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.


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## Abstract Primary effusion lymphoma (PEL) is a rare lymphoma of B‐cell origin, developed in serous cavities. PEL tumor cells are latently infected with Kaposi sarcoma‐associated herpesvirus (KSHV) and in most cases co‐infected with Epstein‐Barr virus (EBV). In 15 primary PEL tumors including 10 E