Association between hemolysis intensity and clinical evolution in patients with sickle cell disease

Abstract

Sickle cell disease (SCD) is a public health matter, especially in sub-Saharan Africa, where there is the largest burden of the disease with about 6 million affected people. Nigeria is the country with the highest burden of disease worldwide. Due to the slave trade and people migration, the disease has spread from Africa to other parts of the world, including Brazil. Although, several hypotheses have been proposed to the clinical diversity of SCD, these factors do not completely explain the phenotypic expressions observed in patients. Therefore, research has been made to search for phenotypic patterns of disease markers. This is the first detailed study of phenotypic correlation with hemolysis markers and genetic polymorphisms involving patients in Brazil. The results of this study will establish a connection between the hypothetical markers and clinical phenotypes of patients with sickle cell disease and it will be useful to predict the severity of the disease and influence therapeutic decisions. In addition, the correlation study of hemolysis can identify which markers are specific to certain complications, and may therefore be useful for the prognosis.