Stroke in adult sickle cell anemia patients.

Abstract

Sickle cell anemia occurred due to a single mutation in beta globin gene and its is characterized by vaso-occlusion episodes (pain crises), vasculopathy, acute thoracic syndrome, ischemic stroke, etc. Strokes are due to macro vascular lesions with vaso-occlusion and had an incidence of 10 to 25%. At present time, to prevent recurrent stroke in pediatric patients, chronic transfusion is used. In adults' patients, there are few data about chronic transfusion and, however some authors believed that transfusions could be substitute by hydroxyurea (HU), the majority of the papers do not confirm this hypotheses. The clinical expertise of the Outpatient clinic of Hereditary Anemias from Escola Paulista de Medicina showed conflicting results, since almost all adult patients who have had stroke are in use of HU with no transfusion and no recurrent strokes. To determine factors associated to this follow up and the actual necessity of chronic transfusion to prevent recurrent stroke in adults, we are going to evaluate retrospective data from 23 patients, such as age of the stroke, beginning of the chronic transfusion, period of treatment with chronic transfusion, date of the transfusion suspension, beginning of HU and period of time of HU use. Moreover, it will be search other associated factors that could interfere in this manifestation such as transcranial and carotidal Doppler, alpha-thalassemia, gene beta haplotypes and single polymorphisms of the genes TNF±, ADCY9, TGFBR3 and IL4R.