Active Von Willebrand Factor in Plasma of Sickle Cell Patients Is An Indicator for Severity of Sickle Cell Anemia

Vaso-occlusion, hemolysis and oxidative stress are hallmarks of sickle cell anemia. We and others have shown that oxidants generated in sickle cell anemia can stimulate endothelial cells to release ultra-large von Willebrand factor (ULVWF), which is hyperactive compared to the normal plasma VWF in binding platelets and red blood cells, and is capable of binding sickle erythrocytes especially well. For this reason, we surveyed several parameters...