Vascular Events Amongst Patients With Sickle Cell Disease

Introduction: Sickle cell disease (SCD) is one of the most common genetic disorders, it not only has hematological pathophysiological consequences, but also has systemic pathological manifestations such as vascular events. It is characterized by structurally abnormal hemoglobin (HbS) that polymerizes to “sickle” red blood cells under stress, most commonly due to hypoxia. SCD can lead to hemolysis and in turn causes endothelial dysfunction and sequestration of sickled cells which can result in cardiovascular or cerebrovascular events (VEs). Furthermore, increased expression of adhesion cellular signaling pathways of red blood cells and endothelial cells and endothelial damage further enhance vascular thrombotic events. Prior studies have described that VEs are associated with poor outcomes in the short term and decreased quality of life in patients over the long term. Data has been lacking in regards to the burden of these VEs in SCD from a population based study. We sought to estimate the trends, predictors and outcomes of VEs in SCD patients from a nationally representative database of the USA.