Human Herpesvirus 8–Unrelated Primary Effusion Lymphoma–Like Lymphoma: Report of a Rare Case and Review of 54 Cases in the Literature

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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.


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


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.


In various aspects, HHV-8–unrelated PEL-like lymphoma is a different entity from HHV-8–related PEL. Immunophenotype, morphology, and c-myc/8q24 status should be included for differential diagnosis. A test for c-myc or 8q24 abnormalities should be recommended for subdividing HHV-8–unrelated PEL-like lymphoma, which may have benefits in patient management.

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