Atherosclerosis and unstable angina in Bernard-Soulier syndrome.

Platelets and von Willebrand factor play pathogenetic roles in atherosclerosis and acute coronary artery ischemic syndromes. Patients with Bernard-Soulier Syndrome are deficient in several platelet membrane glycoproteins, including glycoprotein Ib (GpIb). Glycoprotein Ib is the primary platelet receptor for von Willebrand factor and plays a critical role in the initiation of thrombus formation. Glycoprotein Ib, but also GpIIb/IIIa, mediates the adhesion of platelets to damaged endothelium, particularly at the high shear stresses found in small or diseased arteries. A patient with Bernard-Soulier syndrome is described who developed coronary artery atherosclerosis and unstable angina requiring coronary artery bypass grafting. The implications of this experiment in nature on the contribution of platelets and platelet GpIb and GpIIb/IIIa receptors to the development of atherosclerosis and unstable angina are discussed.