Hypercoagulability and sickle cell disease

Abstract

Thrombotic complications have always been recognized as one of the hallmarks of sickle cell disease (SCD). Epidemiological data demonstrate that stroke and venous thromboembolism are much more frequent in these patients. Furthermore, hypercoagulability has been implicated in the pathogenesis of other complications of SCD such as acute chest syndrome. In the last two decades, robust experimental data have demonstrated that almost every element of hemostasis, both protein and cellular, is altered in a way that shifts the hemostatic balance towards a procoagulant state in SCD. During recent years, exciting new data have shed light on the mechanisms responsible for these alterations, such as infl ammation, endothelial activation, and intravascular hemolysis. In this chapter, we discuss the clinical and laboratory evidence supporting the concept that SCD is associated with a signifi cant hypercoagulable state, as well as the potential mechanisms responsible for these alterations. We also discuss old, current and future therapeutic strategies aimed to modulate the risk of thrombosis in SCD.