Sickle cell disease is an inherited disease characterized by a complex and varied physiopathology. Apart from the sickle red blood cells, responsible for vascular occlusion and hemolytic anemia, changes in the coagulation system seem to play an important role in the clinical manifestations of this disorder. Nearly every component of hemostasis, including platelet function and the procoagulant, anticoagulant, and fibrinolytic systems, are altered in sickle Cell Disease even in non-crisis steady-state patients. The presence of phosphatidylserine on the external membrane of the red blood cell and the increase in antiphosphatidylserine antibodies and tissue factor mark the beginning of coagulation activation in these patients. Moreover, red blood cell destruction decreases the bioavailability of nitric oxide, which modifies hemostasis, increasing platelet activation and the adhesion of molecules to the vascular endothelium. Thus, this study aimed to evaluate several hemostatic changes in patients with sickle cell disease in order to have a better understanding of the "hypercoagulable" state described in this disease.
Blood coagulation; anemia; sickle cell