Classic KSHV/HHV-8-Positive Primary Effusion Lymphoma (Pel): A Systematic Review and Meta-Analysis of Case Reports

Abstract

Primary effusion lymphoma (PEL) is a large B-cell lymphoma growing within body-cavities caused by the Kaposi sarcoma-associated herpesvirus (KSHV)/human herpesvirus-8 (KSHV/HHV-8). It is mainly reported in HIV-infected patients. The uncommon occurrence in the elderly supports a form paralleling classic Kaposi sarcoma (KS), i.e. classic PEL, whose characteristics are relatively underexplored. To better understand the diagnostic modalities and clinical-epidemiological features of classic PEL, articles reporting cases of PEL were identified through MEDLINE/EMBASE databases (January 1998-July 2020) and screened according to PRISMA guidelines to extract individual-level data. A comparison was also performed between classic PEL and classic KS to evaluate similarities and differences. We identified 105 subjects (median age 77 years; 86% males), mainly from Mediterranean countries (52%, first Italy) and Eastern Europe (7%). Common comorbidities were heart failure (32%), cirrhosis (16%), and malignancy (20%) including lymphoid neoplasms. Pleural cavity was the commonest site (67%). PEL diagnosis was based on cytomorphology (89%), evidence of KSHV/HHV-8 infection (94%), EBV co-infection (28%) and clonality of IGH (59%), IGK (14%), TRG (9%) alone or in multiple combinations. Compared to KS, age (P<.001), gender-ratio (P=.08) and mortality (P<.001) were significantly higher in PEL, whereas the frequency of PEL as a second primary was similar (P=.44). This is the first systematic review of classic PEL case reports highlighting heterogeneity and lack of a uniform multidisciplinary approach at diagnosis, in the absence of specific guidelines as it happens for rare cancers. It is conceivable that classic PEL is still underdiagnosed in Mediterranean countries wherein KSHV/HHV-8 is endemic.

Rare Case of Primary Effusion Lymphoma mimicking Pulmonary Tuberculosis

“…Radiologists are essential in verifying the existence of fluid accumulations within body cavities and excluding lymph node enlargement, organ swelling, or any other masses beyond the body cavities to confirm the diagnosis. 5,6 Sophisticated imaging tools such as diffusion-weighted magnetic resonance imaging (DW-MRI) and chest computed tomography (CT) scans are fundamental for accurate diagnosis. They help distinguish between lymphoma subtypes and monitor treatment response effectively.…”

Primary Effusion Lymphoma, Multicentric Castleman’s Disease, and Kaposi’s Sarcoma in an HHV-8 and HIV-Positive Patient: A Case Report

“…PEL is a rare subtype of non-Hodgkin’s lymphoma (NHL) characterized by serous involvement of cavities, causing effusions rather than obvious masses [ 1 ]. Up to 80% of cases occur in individuals with both HIV and Epstein–Barr virus (EBV) infection, but the defining feature of PEL is an infection of the tumor cells via human herpesvirus 8 (HHV-8) [ 1 , 2 ]. Cell morphology can be variable in many cases with plasmacytoid differentiation and characteristically with the loss of B-cell markers such as cluster of differentiation (CD) 20 [ 2 ].…”