CORRELAÇÃO ENTRE OS HAPLÓTIPOS DO GENE DA β-GLOBINA E OS NÍVEIS DE DHL E A NECESSIDADE DO USO DE HIDROXIUREIA EM PACIENTES COM DOENÇA FALCIFORME NO ESTADO DA PARAÍBA

Abstract

Sickle cell anemia is a genetic disease caused by homozygosity of hemoglobin S (Hb SS) and characterized by hemolysis with clinical manifestations that occur due to tissue hypoxia and vaso-occlusive phenomena. The severity of clinical manifestations is attributed to the difference in fetal hemoglobin (HbF) concentration associated with hemoglobin S haplotypes. Other factors may also increase the chance of complications, such as low hemoglobin (Hb) levels and high levels of lactic dehydrogenase. Hydroxyurea is one of the most effective therapies against sickle cell anemia, by inducing increased fetal hemoglobin synthesis, which reduces hemolysis phenomena. This study has the objetive to characterize the correlation between β globin gene haplotypes and DHL levels with disease severity, in addition to correlating the need for the use of hydroxyurea in patients with sickle cell disease. A cross-sectional study with quantitive analysis will be carried out through the collection of retrospective data from children and adolescents aged 0 to 18 years old diagnosed with sickle cell anemia and evaluated by the Hospital Infantil Arlinda Marques in João Pessoa - PB, from March/2020 to August/2020. The research was not carried out because of the pandemic by Covid-19, making it impossible to collect data for the presentation of results and conclusions.