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

7 Citations (Scopus)


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
Issue number3
Publication statusPublished - Mar 2022


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

ASJC Scopus subject areas

  • Clinical Biochemistry


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