Clinical characteristics and survival outcome of primary effusion lymphoma: A review of 105 patients

Abstract

Primary effusion lymphoma (PEL) is a rare and an aggressive B-cell non-Hodgkin lymphoma with a distinctive clinicobiological features. As a result of the rarity of this malignancy, the overwhelming majority of data come from a case reports or series. Our study aimed to evaluate the clinical features and the survival outcomes of 105 patients with PEL who diagnosed between 2001 and 2012 from the Surveillance Epidemiology and End Results database 18 of the US National Cancer Institute. The Kaplan-Meier curves were constructed and the multivariate Cox model was built to identify survival prognostic factors. Of the total 105 patients, the median age at diagnosis was 41 years (male-to-female ratio, 8.5:1), and Caucasian race accounted for 79%. Regarding the diagnostic methods, diagnosis has been established by histology in 39 patients (36.8%) and cytology in 54 patients (51.4%). Ann Arbor staging showed that 65% of the patients had advanced stages (stages III and IV) at time of diagnosis. With a median follow-up of 4 months, 82 patients (78%) had died at last follow-up time, of which 40 patients (58.5%) died due to human immunodeficiency virus (HIV)-related complications and 21 patients (25.6%) due to PEL progression. The HIV-related death accounted for 69% of deaths in a black American compared with 55.5% in Caucasian. With a median overall survival (OS) of 4.8 months, the 1-, 3-, and 5-year OS rates were 30%, 18%, and 17%, respectively. In univariate analysis, age, gender, marital status, and year of diagnosis had no impact on OS. The 1-year OS rate was 25% in advanced stages compared with 52% in early stages. In a multivariate model adjusted for demographic data, the risk of mortality in advanced stages (stages III and IV) was 1.86-fold higher than those reported in early stages (Hazard Ratio, 1.8; 95% confidence interval, 1.05-3.29; P = .03). In conclusion, PEL has a poor survival regardless, age, gender, marital status, and tumor stage.

Keywords: HHV-8; HIV; Primary effusion lymphoma (PEL); SEER; Survival.