Perfil das hemoglobinopatias diagnosticadas em ambulatório no Instituto de Hematologia e Hemoterapia do Amapá (HEMOAP)

Abstract

Hemoglobinopathies are the most common monogenic recessive diseases, with the sickle cell diseases being the most important due to their frequency and morbidity. Folic acid, Penicillin V, hydroxyurea and blood transfusions are used in the treatment. This study aims to verify the most diagnoses hemoglobinopathies Hematology and Hematology Amapá´s Institute mais diagnosticas no Instituto de Hematologia e Homoterapia do Amapá over the years of 1980 to 2017. A total of 132 HEMOAP´s patients data, corresponding to hemoglobinopathies diagnosed by electrophoresis from 1980 to 2017, were analyzed. Hemoglobinopathy, sex, current age and diagnosis, number of deaths and absconding, and the proposed treatment were verified. Of 132 registries, the majority were male (53.04%) and the predominant disease was sickle cell anemia (78.07%). Current age was 6 months to 55 years and the mean at diagnosis was 9.6 years. From total of records, 18 corresponded to deaths and of the 114 alive, 19 evaded. Drug treatment was the most used (69.5%). Different other Brazilian states the predominance of hemoglobinopathies in males and late diagnosis are probably due to the most of Amapá´s population being male and the delay in neonatal screening test delay of implantation, respectively. However, HEMOAP´s data corroborate with other researches by the predominance of sickle cell anemia, predominant drug treatment and the occurrence of deaths with an average of up to 30 years. Because they are chronic and systemic, hemoglobinopathies are a public health problem and require early diagnosis, adequate treatment and continuous follow-up aiming to improve life quality and decreasing of morbidity and mortality.