Bernard-Soulier syndrome /bernär″so͝olyā″/ [Jean A. Bernard, French hematologist, 1907–2006; Jean-Pierre Soulier, French hematologist, 1915–2003] , an autosomal-recessive bleeding disorder characterized by an absence of or a deficiency in the ability of the platelets to adhere to von Willebrand factor because of the relative lack of membrane glycoprotein Ib/V/IX. On microscopic examination the platelets appear large and gray. The use of aspirin may provoke hemorrhage. After trauma or surgery, loss of blood may be greater than normal and a transfusion may be required.
