Sickle cell anemia: delayed diagnosis in Bahia, Brazil--a largely Afro-descendant population

Hemoglobinopathies are the most common genetic disorders in the world and include sickle cell anemia (SCA), which is a public health problem in Brazil. Nevertheless, the disease is highly unknown among health professionals, and delayed diagnosis constitutes an important cause of concern for caretakers of SCA patients. The purpose of this study was to compare the clinical and laboratory history of SCA patients whose diagnosis was established during the first year of life to those of other SCA patients who had delayed SCA diagnosis. Demographic, clinical, and laboratory data were all reviewed from 99 steady-state SCA patients who were followed in a public hematology and hemotherapy clinic in Salvador, Brazil. The patients were aged > or = 12 years and attended the outpatient unit at least once from November 2008 to June 2009. The data were analyzed in 2010. For all patients, the mean age (+/- SD) at diagnosis was 12.7(+/- 12.1) years, ranging from 0 to 47 years. Mean age was higher in patients whose SCA diagnosis was established after age 5 (32.9 +/- 11.9 years, P = .005). Increased unconjugated bilirubin, stroke and splenic sequestration were more prevalent in patients who were diagnosed in the first year of life (P = .043, .024 and .026 respectively). The data suggest that stroke, splenic sequestration and unconjugated bilirubin level may be influenced by age at SCA diagnosis.