A Case of HIV-Negative Primary Effusion Lymphoma Treated with Bortezomib, Pegylated Liposomal Doxorubicin, and Rituximab

Primary effusion lymphoma (PEL) is a rare B-cell non-Hodgkin lymphoma that usually occurs in HIV-positive patients and has a very poor prognosis with limited treatment options. Prospective studies are lacking to define the standard of care for this disease, and various case series report median survival at 6 months with anthracycline-based chemotherapy. Reports of antiviral agents, immune modulators, and some targeted therapies are present in the literature with variable results. Herein, we report a case of an elderly HIV-negative man of Mediterranean origin who was diagnosed with primary effusion lymphoma and responded dramatically to 6 cycles of a combination of bortezomib, pegylated liposomal doxorubicin, and rituximab. He has since been maintained on rituximab and remains in complete remission 2 years after diagnosis. In this report, we discuss the rationale for using these agents in this patient and advocate the further study of bortezomib-based therapy in PEL.