Primary effusion lymphoma in human immunodeficiency virus (HIV)–negative population: A systematic review and meta-analysis.

Abstract

e19089 Background: Primary effusion lymphoma (PEL) represents a small proportion of non-Hodgkin lymphomas, historically associated with human immunodeficiency virus (HIV) infection. Despite this, multiple reports have been made of cases of PEL among HIV-negative patients, particularly those who have previously undergone solid organ or hematopoietic cells transplant. Data remains limited regarding the impact and outcomes of HIV-negative patients with primary effusion lymphoma. We sought to perform a systematic review and meta-analysis on impact of PEL among HIV-negative patients. Methods: We performed a systematic search in Medline and Embase, following PRISMA guidelines. Observational studies describing the proportion of non-HIV patients in a cohort of primary effusion lymphoma (PEL) patients were included. Case reports, case series with less than 10 patients, and studies from national database cohorts that could overlap with other studies’ population were excluded. Data was collected for the outcomes of interest (primary endpoint proportion of HIV negative patients, and secondary endpoints were number of HIV-negative patients with prior history of transplant, 1-year overall survival, 5-year overall survival, percentage of HIV-negative patients with single cavity involvement, percentage of HIV-negative patients with extra-cavitary disease and rate of other Kaposi sarcoma-related herpesvirus (KSHV) associated diseases. A single-arm proportion meta-analysis for the outcomes of interest was performed in R studio version R 4.4.2. A random effects model was used considering the high heterogeneity of observational studies. Results: We screened 4384 studies, and 3 studies met inclusion criteria (sample size range 19-70). The total cohort of patients was 114 patients. Male sex was predominant in all studies included (88-95.7%). The median age among non-HIV patients was reported in two studies, ranging between 70 and 81.9 years. The proportion of HIV-negative patients among all PEL diagnosis was 25% (95%-CI 10-50). Among HIV-negative patients, just two cases of previous transplant were reported (25% of HIV-negative patients of the study in question). The 1-year overall survival (OS) rate was 50% (95%-CI 30-70). Two studies reported 5-year OS, and it was 0% in both cohorts. Rates of single cavity involvement varied between 44-87.5%, and rate of extra-cavitary disease was reported only in one study (12.5%). Conclusions: Patient without HIV infection represent a significant proportion of the population affected by primary effusion lymphoma and have a high mortality rate at 1 and 5 years follow up. Despite its impact, data regarding outcomes of PEL in HIV-negative patients remains scarce, and heterogenous among current studies. Further studies are required for appropriate characterization of PEL outcomes in HIV-negative patients.