Prevalence of sickle cell disease in adults with delayed diagnosis

Abstract

Objective: To estimate the prevalence of sickle cell disease in adults with delayed diagnosis, receiving treatment at hematology outpatient clinics in the health network of the state of Mato Grosso do Sul, between 2013 and 2017; to describe sociodemographic characteristics; to verify associations between genotypes in relation to current age, the genotypes, and age at diagnosis. Methods: A retrospective, cross-sectional study with data collected at two teaching hospitals. The variables investigated were: year of treatment, genotype, sex, date of birth, age at diagnosis, and city in which they lived. Prevalence was estimated per point, using a 95% confidence interval. Results: The prevalence was 3.9% in 103 adults with sickle cell disease: 60 female and 43 male. The HbSS genotype was predominant, followed by HbSC. The median age was 35 for HbSS, and 31 for HbSC. Median age at diagnosis was five years for HbSS, and 21 for HbSC. No association was found between age (years) of patients and genotype (chi-square test p=0.601), or between genotype and age group (chi-square test p= 0,318). Conclusion: The most frequent genotype was HbSS, followed by HbSC. The diagnosis of patients with hemoglobin SC occurred later in life than those with the hemoglobin SS genotype. Sociodemographic variables and delayed diagnosis warns for the need to strengthen actions in the health network, which interfere significantly in the morbidity and mortality of adults with sickle cell disease.