Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018

PURPOSE: To analyze the clinical-epidemiological characteristics and mortality in patients with sickle-cell anemia (SCA). PATIENTS AND METHODS: A cohort study with retrospective data, conducted in two reference hospitals for SCA treatment from January 1980 to December 2018, recorded in two reference services. With a 5% significance level, the Chi-Square and Student’s t-tests were employed in the inferential statistical analysis. RESULTS: A total of 128 patients with SCA were studied. Diagnosis up to the fifth day of life was made in 10 patients. There were 19 deaths, of which 12 (63.2%) were female, and the average age at death was 27.05 (± 14.78) years. The leading causes of death were septic shock and cardiogenic shock. The use of invasive medical devices was considered a risk factor for death (RR=2.63; 95% CI=1.16–5.96; p=0.018), and monitoring time up to 20 years presented a 31% reduction in the risk of dying (RR=0.31; 95% CI=0.12–0.82; p=0.011) when compared to the monitoring of more than 20 years. CONCLUSION: These findings are to be considered in the treatment of patients with SCA, mainly regarding early diagnosis and access to the treatment immediately afterward, since they are fundamental in improving survival and reducing severe complications.