Prothrombotic aspects of sickle cell disease

Abstract

Sickle cell disease (SCD) is a hematologic disorder caused by a well-characterized point mutation in the β-globin gene. Abnormal polymerization of hemoglobin tetramers results in the formation of sickle red blood cells that leads to vascular occlusions, hemolytic anemia, vascular inflammation and cumulative, multiple organ damage. Ongoing activation of coagulation is another hallmark of SCD. Recent studies strongly suggested that hypercoagulation in SCD is not just a secondary event but contributes directly to the disease pathophysiology. In this article we summarize mechanisms leading to the activation of coagulation, review data indicating direct contribution of coagulation to the pathology of SCD and, we discuss the anticoagulation as a possible treatment strategy to attenuate the disease progression.

Keywords: PAR; coagulation; sickle cell disease; thrombosis; tissue factor.