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The clinical picture of patients with sickle cell anemia (SCA) is associated with several complications some of which could be fatal. The objective of this study is to analyze the causes of death and the effect of sex and age on survival of Brazilian patients with SCA. Data of patients with SCA who were seen and followed at HEMORIO for 15 years were retrospectively collected and analyzed. Statistical modeling was performed using survival analysis in the presence of competing risks estimating the covariate effects on a sub distribution hazard function. Eight models were implemented, one for each cause of death. The cause?specific cumulative incidence function was also estimated. Males were most vulnerable for death from chronic organ damage (p = 0.0005) while females were most vulnerable for infection (p=0.03). Age was significantly associated (p ? 0.05) with death due to acute chest syndrome (ACS), infection, and death during crisis. The lower survival was related to death from infection, followed by death due to ACS. The independent variables age and sex were significantly associated with ACS, infection, chronic organ damage and death during crisis. These data could help Brazilian authorities strengthen public policies to protect this vulnerable population.