Cancer Res Treat.  2019 Oct;51(4):1302-1312. 10.4143/crt.2018.555.

Body Cavity–Based Lymphoma in a Country with Low Human Immunodeficiency Virus Prevalence: A Series of 17 Cases from the Consortium for Improving Survival of Lymphoma

• Shin J
• Ko YH
• Oh SY
• Yoon DH
• Lee JO
• Kim JS
• Park Y
• Shin HJ
• Kim SJ
• Won JH
• Yoon SS
• Kim WS
• Koh Y
• On behalf of the Consortium for Improving Survival of Lymphoma investigators

• KMID: 2460580
• DOI: http://doi.org/10.4143/crt.2018.555

Abstract

PURPOSE
Primary effusion lymphoma (PEL) is a type of body cavity-based lymphoma (BCBL). Most patients with PEL are severely immunocompromised and seropositive for human immunodeficiency virus (HIV). We investigated the distinctive clinicopathologic characteristics of BCBL in a country with low HIV burden.
MATERIALS AND METHODS
We retrospectively collected data on the clinicopathologic characteristics, treatments, and outcomes of 17 consecutive patients with BCBL at nine institutions in Korea.
RESULTS
Latency-associated nuclear antigen 1 (LANA1) immunostaining indicated that six patients had PEL, six patients had human herpesvirus 8 (HHV8)-unrelated BCBL, and five patients had HHV8-unknown BCBL. The patients with PEL exhibited no evidence of immunodeficiency except for one who was HIV positive. One (20%) and four (80%) patients with PEL and six (100%) and zero (0%) patients with HHV8-unrelated BCBL were positive for CD20 and CD30 expression, respectively. The two patients with PEL (one HIV-positive and one HIV-negative patient) with the lowest proliferation activity as assessed by the Ki-67 labeling index survived for > 1 and > 4 years without chemotherapy, respectively, in contrast to the PEL cases in the literature, which mostly showed a high proliferation index and poor survival.
CONCLUSION
PEL mostly occurred in ostensibly immunocompetent individuals and had a favorable outcome in Korea. A watchful waiting approach may be applicable for managing HIV-seronegative patients with PEL with a low Ki-67 labeling index. A possible trend was detected among LANA1, CD20, and CD30 expression in BCBL.

Keyword

Body cavity-based lymphoma; Primary effusion lymphoma; Human herpesvirus 8; Human immunodeficiency virus; Immunophenotype

MeSH Terms

Drug Therapy
Herpesvirus 8, Human
HIV*
Humans*
Korea
Lymphoma*
Lymphoma, Primary Effusion
Prevalence*
Retrospective Studies
Watchful Waiting

Figure

• 

  Fig. 1. Lymphoma cells with positive nuclear staining for latency-associated nuclear antigen 1 in the ascitic fluid of a representative primary effusion lymphoma patient. (A) Ascites smear exhibits individually scattered tumor cells with an immunoblastic or plasmablastic cytomorphology. (B) Immunohistochemistry using an anti‒human herpesvirus 8 antibody shows dark brown nuclear staining of tumor cells.

• 

  Fig. 2. Immunophenotypic analysis and in situ hybridization for Epstein-Barr virus–encoded small RNA (EBER). The cases are presented in the same order as in Table 1. Latency-associated nuclear antigen 1 (LANA1) and EBER are shown in the first and second columns, respectively, while the remaining markers are arranged in descending order of the number of cases in which they were analyzed. All markers that were stained in at least one case are shown. Blank tiles indicate that assays were not performed or data were not available. MUM1, multiple myeloma oncogene 1; IRF4, interferon regulatory factor 4; κ, immunoglobulin κ light chain; λ, immunoglobulin λ light chain.

Reference

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