Hemostatic alterations in sickle cell disease: Relationships to disease pathophysiology

The protean manifestations of sickle cell disease ( SCD), especially, micro vessel involvement in the The vaso-occlusive process, is classically ascrib ed to the phenomena of erythrocyte sickling and enhanced red cell-endotheli al adherence. Pertubations in various hemostatic systems occurs in SCD, bot h in steady state and during vaso-occlusion, with the intravascular generat ion of thrombin. The etiology(s) of thrombin generation in SCD will be desc ribed. Whether the activation of the cellular and plasmatic phases of hemos tasis is causative or occurs as a result of vascular injury will be discuss ed.