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Mortality from sickle cell disease in Brazil.

Paula Fraiman Blatyta Caselli1, Claudia Di Lorenzo Oliveira2, Isabel Gomes3

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Summary

Sickle cell disease patients in Brazil face premature death, with infections and organ damage as leading causes. Key mortality predictors include older age, iron overload, and hospital admissions, necessitating targeted interventions.

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Area of Science:

  • Hematology
  • Public Health
  • Epidemiology

Background:

  • Sickle cell disease (SCD) remains a significant health concern, with mortality rates higher than the general population despite medical advancements.
  • In Brazil, understanding SCD mortality patterns is crucial for public health strategies.

Purpose of the Study:

  • To evaluate the causes and independent predictors of mortality in a Brazilian SCD cohort.
  • To identify factors contributing to premature death in individuals with sickle cell disease.

Main Methods:

  • Analysis of the multicenter REDS-III cohort data (2013-2018) including 2,793 SCD patients.
  • Comparison of deceased and surviving patients using statistical tests and multivariable Cox regression to identify mortality predictors.
  • Confirmation of deaths via chart review and national death certificate linkage.

Main Results:

  • A total of 159 deaths (5.7%) occurred, with a median life expectancy of 65.7 years.
  • Infection (33.3%), non-infectious pulmonary conditions (25.2%), and neurologic disease (14.5%) were leading causes of death.
  • Independent predictors of mortality in adults included older age, iron overload, and prior hospital admissions.

Conclusions:

  • Mortality in Brazilian SCD patients occurs approximately 10 years earlier than in the general population.
  • The burden of SCD mortality is increasingly affecting adults, particularly in their third and fourth decades.
  • Prompt management of infections, acute chest syndrome, stroke, iron overload, and frequent hospitalizations is critical for improving survival in SCD.