Primary effusion lymphoma in Taiwan shows two distinctive clinicopathological subtypes with rare human immunodeficiency virus association
Journal
Histopathology
Journal Volume
72
Journal Issue
6
Pages
930-944
Date Issued
2018
Author(s)
Chen B.-J.
Wang R.-C.
Ho C.-H.
Huang W.-T.
Yang S.-F.
Hsieh P.-P.
Yung Y.-C.
Lin S.-Y.
Hsu C.-F.
Su Y.-Z.
Kuo C.-C.
Chuang S.-S.
Abstract
Aims: To investigate the clinicopathological and molecular features of primary effusion lymphoma (PEL) in Taiwan and the association with human immunodeficiency virus (HIV), human herpesvirus 8 (HHV8) and Epstein–Barr virus (EBV). Methods and results: We investigated retrospectively 26 cases with a median age of 76.5. Only one (4%) patient was infected with HIV. Cytologically, all lymphoma cells revealed typical immunoblastic to plasmablastic morphology. Immunohistochemically, HHV8 was positive in eight (32%) tumours and negative in 17 (68%) cases. All 23 tested cases examined were of the non-germinal-centre B cell phenotype. MYC proto-oncogene (MYC) and Epstein–Barr encoding mRNA (EBER) were positive in 43% (nine of 21) and 17% (four of 23) cases, respectively. Immunoglobulin heavy chain (IGH), B cell lymphoma (BCL)2, BCL6 and MYC were rearranged in 71%, 11%, 12% and 18% cases, respectively. By univariate analysis, the overall survival (OS) was associated statistically with MYC expression (P?=?0.012) and BCL2 rearrangement (P?=?0.035), but not with the others. By multivariate analysis, no factor was statistically significant. Compared to the HHV8-negative cases, the HHV8-positive cases were mainly of the plasmablastic immunophenotype expressing CD30 and CD138, and with a less frequent expression of pan-B cell markers. Conclusions: Apart from the phenotypical difference, our HHV8-positive neoplasms were not distinct from the HHV8-negative group. Literature review of 256 cases, including our cases, revealed that HHV8-positive cases were associated more frequently with HIV and EBV infection, with rare MYC rearrangement, and a poorer prognosis than HHV8-negative cases. We propose to name the HHV8-positive cases as ‘classical’ or ‘type I PEL’ and the HHV8-negative cases as ‘type II PEL’, stressing the similarities and the distinctive features between these two groups. ? 2017 John Wiley & Sons Ltd
Subjects
effusion-based lymphoma; Epstein–Barr virus; fluorescence in-situ hybridisation; human herpesvirus 8; primary effusion lymphoma; Taiwan
SDGs
Other Subjects
immunoglobulin heavy chain; Myc protein; protein bcl 2; protein bcl 6; syndecan 1; tumor necrosis factor receptor superfamily member 8; virus messenger RNA; adult; aged; antigen expression; Article; B lymphocyte; cancer morphology; cancer prognosis; clinical article; controlled study; cytology; cytopathology; diagnostic imaging; Epstein Barr virus; female; human; human cell; Human herpesvirus 8; Human immunodeficiency virus; Human immunodeficiency virus infected patient; human tissue; immunoblastic lymphoma; immunohistochemistry; immunophenotyping; lymphoma cell; male; middle aged; multivariate analysis; overall survival; phenotype; plasmablastic lymphoma; primary effusion lymphoma; priority journal; protein expression; retrospective study; serology; Taiwan; univariate analysis; very elderly; complication; Epstein Barr virus infection; herpes virus infection; Human herpesvirus 8; Human immunodeficiency virus infection; pathology; primary effusion lymphoma; Taiwan; virology; Aged; Aged, 80 and over; Epstein-Barr Virus Infections; Female; Herpesviridae Infections; Herpesvirus 8, Human; HIV Infections; Humans; Lymphoma, Primary Effusion; Male; Middle Aged; Retrospective Studies; Taiwan
Publisher
Blackwell Publishing Ltd
Type
journal article