Splenic Marginal Zone Lymphoma (SMZL) - Outcomes, Prognostic Factors and Risk-Adapted Therapy in Resource-Poor Settings: Data from a Latin American Retrospective Cohort

Introduction: Splenic marginal zone lymphoma (SMZL) is a rare B-cell lymphoid malignancy with primary involvement of the white pulp of the spleen. It is a chronic disease, of indolent behavior and with prolonged survival. However, approximately 25% of cases have greater biological aggressiveness, a propensity for histological transformation to high-grade B-cell non-Hodgkin's lymphoma and shortened survival. The recognition of these cases of particular outcome is important for the selection of a risk-adapted therapeutic approach in resource-poor settings. Methods: In this single-center, retrospective and observational study, we describe the clinical and epidemiological characteristics, analysis of survival, determination of prognostic factors and propose a risk-adapted therapeutic approach, based on data from a cohort of 39 SMZL patients, treated in the largest cancer center in Latin America, from January 1992 to December 2016. Factors with prognostic implications were determined by Cox's univariate analysis, survival curves were estimated by the Kaplan-Meier method and the log-rank test was used to compare curves of survival and to verify the association between categorical variables and survival curves. Results: The clinical and epidemiological characteristics of the 39 SMZL patients are summarized inTable 1. There was predominance of female patients (71.8% - 28-29), with a median age of 63 years (28-76) and a higher incidence of B symptoms (56.4% - 22-39) and extra-splenic involvement (87.1% - 34-39) than the results described in series in Europe and North America. With a median follow-up of 8.7 years (0.6-20.2 years), the overall survival (OS) estimated at 5 years and progression-free survival (PFS) were 76.9% and 63.7%, respectively. Factors with an adverse prognostic impact on OS included: LDH ≥ 480 UL (HR: 4.55, 95% CI 1.05-19.70, p = 0.043), albumin Conclusion: Therefore, in resource-poor settings, where access to anti-CD20 immunotherapy is not universal for patients with SMZL, we suggest that the first-line treatment should consist of monotherapy with rituximab for elderly patients, with high surgical risk or with at least one risk factor identified in our study. Remainders can be safely managed with splenectomy. Download : Download high-res image (382KB) Download : Download full-size image Disclosures No relevant conflicts of interest to declare.