Abstract
Abstract Sickle cell disease (SCD) is described as a hypercoagulable state. Patients exhibit increased platelet activation, high plasma levels of markers of thrombin generation, depletion of natural anticoagulant proteins, abnormal activation of the fibrinolytic system, and increased tissue factor expression, even in the noncrisis “steady state.” Furthermore, SCD is characterized by an increased risk of thrombotic complications. The pathogenesis of coagulation activation in SCD appears to be multifactorial, with contributions from ischemia-reperfusion injury and inflammation, hemolysis and nitric oxide deficiency, and increased sickle RBC phosphatidylserine expression. Although recent studies in animal models of SCD suggest that coagulation activation may contribute to the pathogenesis of SCD, the data on the contribution of coagulation and platelet activation to SCD-related complications in humans are limited. In this chapter, we review selected complications that may be associated with thrombosis as well as currently available treatments for SCD, including hydroxyurea and red blood cell transfusion. In addition, we review the available data on anticoagulants and antiplatelet agents in patients with SCD.
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