Primary Effusion Lymphoma: A Timely Review on the Association with HIV, HHV8, and EBV

Chih Yi Liu, Bo Jung Chen, Shih Sung Chuang

Research output: Contribution to journalReview articlepeer-review

Abstract

Primary effusion lymphoma (PEL) is defined by the WHO classification as a large B-cell neoplasm without detectable tumor masses. It is universally associated with HHV8, with most cases occurring in the setting of immunodeficiency such as HIV infection, and a poor prognosis. Morphologically, the neoplastic cells range from immunoblastic, plasmablastic, to anaplastic; and phenotypically, most cases express plasma cell but not B-cell markers, i.e., plasmablastic. During the past decade, primary HHV8-negative effusion lymphoma has been reported. Such cases were considered in the WHO classification scheme as effusion-based lymphoma. We performed a systemic review of 167 HHV8-negative effusion lymphomas from the literature and found that only 42% were associated with a fluid overload state, and with low rates of HIV (6%) or EBV (21%) infection. Furthermore, most patients are old (or immunosenescent) with underlying medical conditions/comorbidities, most neoplasms are of B-cell phenotype, and the outcome is more favorable than that of HHV8-positive PEL. These distinctive findings supported our prior proposal of designating these HHV8-negative cases as type II PEL, in contrast to the classic or type I PEL as defined by the WHO. Furthermore, we propose an algorithmic approach for the diagnosis of PEL and its mimickers.

Original languageEnglish
Article number713
JournalDiagnostics
Volume12
Issue number3
DOIs
Publication statusPublished - Mar 2022

Keywords

  • EBV
  • effusion-based lymphoma
  • HHV8
  • HIV
  • primary effusion lymphoma

ASJC Scopus subject areas

  • Clinical Biochemistry

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