β-Thalassemia is an autosomal recessive inherited blood disorder caused by defects in the production of globin chains, which combine to form hemoglobin. β-Thalassemia strongly influences the daily lives of affected people, by determining important psychological and cognitive sequelae: cognitive deficits can emerge early during development with long-term consequences for quality of life. Although the literature underlies the presence of various cognitive impairments in β-Thalassemic people, recent studies have mostly investigated the presence of intellectual disability, while only a small part focused on specific cognitive functions. The aim of this systematic review is to identify different cognitive profiles (depending on type of β-Thalassemia and age) and the relation between these profiles and sociodemographic variables, psychological and clinical factors in order to inform multidimensional assessments and to implement interventions focused on the promotion of cognitive health across the life span in β-Thalassemic people. Current evidence suggests that neuropsychological assessment should be an integral part of the diagnostic process, highlighting the need for early neuropsychological interventions in order to improve the quality of life. The results are also discussed in light of cultural differences and guidelines of treatment.
Keywords: cognitive profiles; cognitive sub-domains; neuropsychological assessment; β-Thalassemia.