Human herpesvirus 8-unrelated primary effusion lymphoma-like lymphoma: report of a rare case and review of 54 cases in the literature.

Objectives: To report a patient with primary effusion lymphoma who was negative for human herpesvirus-8 (HHV-8), human immunodeficiency virus, Epstein-Barr virus, hepatitis C virus, and hepatitis B virus, as well as review 54 reported cases of HHV-8-unrelated primary effusion lymphoma (PEL)-like lymphoma in the literature to clarify the nature of this entity.

Methods: The patients' characteristics, clinical presentation, pathogenesis, morphologic-immunophenotypic features, clinical management, and prognosis were studied.

Results: HHV-8-negative PEL-like lymphomas often occur in immunocompetent and elderly patients, are sometimes associated with chronic inflammation-related fluid overload, are mostly large B-cell or large B-cell with plasmacytic differentiation type, and are associated with a better prognosis.