Hemoglobin SC disease and its clinical manifestations in pediatric patients: where is the data?

Abstract

Hemoglobin SC Disease is among the main hemoglobinopathies related to sickle cell anemia (only surpassed in prevalence by Hemoglobin SS Disease). Although considered a pathology of lesser severity and complications, information on its clinical manifestations seem to lack support in consistent studies. In the pediatric population, data is even more scarce. The present study carried out an integrative review of the clinical manifestations reported in pediatric patients with hemoglobin SC disease, with their age of occurrence, the most prevalent manifestations, evolution of pathology and associated morbidity. The articles analyzed were able to provide guidance on the issues examined, but the data lacked consistency. New and greater number of prospective studies are necessary to increase the level of evidence for the clinical findings presented in this entity.