[Bernard-Soulier syndrome from the clinical description (1948) to the molecular era (1977) (author's transl)].

Abstract

Bernard-Soulier syndrome is a constitutional thrombopathy with an impaired platelet adhesion to the vessel wall. Since the first description in 1948 many works had been reported, and recently a molecular abnormality of the platelet membrane was shown. Interactions between specific membrane sites and platelet functions are now proposed.