Bernard-Soulier syndrome (BSS), also known as Hemorrhagiparous thrombocytic dystrophy, is a hereditary bleeding disorder affecting the megakaryocyte/platelet lineage and characterized by bleeding tendency, giant blood platelets and low platelet counts. T
Bernard-Soulier syndrome (BSS), also known as Hemorrhagiparous Thrombolytic Dystrophy, is an extremely rare inherited blood clotting disorder characterized by giant platelet cells, thrombocytopenia, and prolonged bleeding time. In 1948, Jean-Bernard and Jean-Pierre Soulier described the first male patien...
Point mutation in a leucine-rich repeat of platelet glycoprotein Ibα resulting in the Bernard-Soulier-Syndrome J Clin Invest., 92 (1993), pp. 1213-1220 View in ScopusGoogle Scholar 20. Shattil SJ, Kashiwagi H, Pamporri N Integrin signaling: the platelet paradigm Blood., 91 (1998), pp....