Hemostatic alteration in sickle cell disease: Pathophysiology of the hypercoagulable State

Abstract

Sickle cell disease (SCD) is a monogenic genetic disease inherited in an autosomal recessive manner and distinguished by the presence of defective hemoglobin, known as homozygous sickled hemoglobin disease (HbSS). Sickled red blood cells lead to blood vessel obstruction, hemorrhage, and critical hemostatic function alterations. Defective hemoglobin that associated with serious health problems, such as thromboembolism among SCD patients, is clearly documented. Empirical evidence indicates that hypercoagulability states and proinflammatory phenotypes in patients with SCD are a substantial contribution of thromboembolic complications, with promoting morbidity and mortality. This review discusses the involvement of vascular endothelial cell, platelet, and coagulation cascade in the thrombogenesis of SCD.