Resumen:
he antiphospholipid syndrome (APS) is characterized by recurrent thromboembolic events in the setting of pathologic autoantibodies, some of which are directed to β2-Glycoprotein 1 (β2GPI). The mechanisms of thrombosis in APS appear to be, multifactorial, and likely includes a component of endothelial activation. Among other things, activated endothelium secretes von Willebrand factor, a hemostatic protein that in excess can increase the risk of thrombosis.We hypothesized that anti-β2GPI antibodies could regulate the release and modulation of VWF from endothelial cells. We observed that anti-β2GPI antibodies from some patients with APS induced VWF release from human endothelial cells but did not induce formation of cell-anchored VWF-platelet strings. Finally, we also determined that one of the Anti-β2GPI antibodies tested can inhibit the function of ADAMTS13, the main modulator of extracellular VWF. These results suggest that VWF and ADAMTS13 may play a role in