Bernard Soulier syndrome (BSS) is an inherited platelet disorder characterized by mild to severe bleeding tendency, macrothrombocytopenia and absent ristocetin-induced platelet agglutination. The disease is Bernard-Soulier syndrome (Monarch Disease Ontology entry MONDO_0009276). Also known as: Bernard - Soulier thrombopathy, Bernard Soulier syndrome, Bernard-Soulier syndrome, type A1 (recessive), Hemorrhagiparous thrombocytic dystrophy, giant platelet disorder, isolated, giant platelet syndrome, hemorrhagic dystrophic thrombocytopenia, thrombopathy, Bernard-Soulier.