Síndrome da encefalopatia posterior reversível associada à transfusão em anemia falciforme: relato de caso

Abstract

Sickle Cell Anemia (SCA) is the most common genetic disease in the world, characterized by frequent vaso-occlusive crises that, along with extensive pathophysiology, predispose individuals to neurological events such as Stroke. Posterior Reversible Encephalopathy Syndrome (PRES), on the other hand, is an uncommon manifestation resulting from vasogenic edema whose pathogenesis is not completely understood. Individuals with hemoglobinopathies have a higher chance of developing PRES; however, there are few reports in the literature on the occurrence of PRES related to SCA. The aim of the study is to report a case in which red blood cell transfusion (RBCT) was followed by PRES in a child with SCA. This is a longitudinal single-arm study with systematic research in major databases using the PICO strategy to associate the three events (SCA, RBC, PRES) with their appropriate medical descriptors. The present study evidenced most findings described in the scientific literature, reinforcing the need to expand knowledge of PRES as a differential diagnosis of neurological events in hemoglobinopathies in order to improve healthcare quality and reduce morbidity and mortality in these individuals