Human Herpesvirus 8–Unrelated Primary Effusion Lymphoma–Like Lymphoma

Report of a Rare Case and Review of 54 Cases in the Literature

Wu, William MD, PhD
Youm, Wonita MD
Rezk, Sherif A. MD
Zhao, Xiaohui MD, PhD

Department of Pathology and Laboratory Medicine, University of California, Irvine

Address reprint requests to Dr Zhao: Dept of Pathology, University of California Irvine Medical Center (UCI), 101 The City Dr, Orange, CA 92867; e-mail: [email protected].

American Journal of Clinical Pathology 140(2):p 258-273, August 01, 2013. | DOI: 10.1309/AJCPHZ3CHO4HUWET

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.

Conclusions:

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.