Increased prothrombotic risk in SCD is linked to elevated TSP-1 levels, which not only inhibit ADAMTS13 proteolysis of von Willebrand Factor [55], but also provoke RBC MP shedding; this process, in turn, favors RBC adhesion to endothelial cells as well as stimulation of endothelial cell apoptosis [56]. The gene discussed is ADAMTS13; the disease is Schnyder corneal dystrophy.