Primary effusion lymphoma (PEL) is recognized as a unique clinicopathological entity associated with human herpesvirus 8 (HHV-8), and it occurs almost exclusively in human immunodeficiency virus (HIV)-infected individuals. In the majority of PEL cases, Epstein-Barr virus (EBV) has been found in the
tumor cells as well. We describe here an elderly HIV seronegative female patient with PEL in the pleura and pericardium not associated with HHV-8 or EBV. Cytologic examinations of the
pleural effusion revealed large
lymphoma cells with immunophenotypes positive for CD8, CD10, CD19, CD20, CD22, CD24, CD45, and
HLA-DR but negative for CD30 and
surface immunoglobulin. Chromosome analysis showed complicated abnormalities including add(3)(q27). Immunoglobulin gene rearrangement was detected by Southern blotting; however, c-myc, Bcl-2, and Bcl-6 genes were not rearranged. The patient was treated with a modified CHOP (
cyclophosphamide, hydroxydoxorubicin,
oncovine, and
prednisolone) regimen, and achieved remission. Recurrence of PEL in the pericardium as effusion
lymphoma was found 3 months after the discontinuation of CHOP. After approximately 1 year of intermittent multiagent
salvage therapy for pericardial recurrences, a treatment that resulted in a partial response, 3 cycles of monotherapy with
sobuzoxane were administered. At the time of this report the patient had been free from PEL for more than 18 months without
chemotherapy.