Bernard-Soulier syndrome

Bernard-Soulier syndrome

Blood and lymphoreticular system

Traumatic, mechanical, and vascular disorders

Key Takeaways

Bernard-Soulier syndrome (BSS) is a rare congenital bleeding disorder characterized by thrombocytopenia, unusually giant platelets, and prolonged bleeding time. Patients with BSS typically have prolonged or severe bleeding after minor trauma or surgery and may also develop epistaxis, hematomas, and petechiae. The severity of the condition can vary from mild to life-threatening.