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Bernard-Soulier syndrome

pathology

Quick Summary

Bernard-Soulier syndrome is an inherited bleeding disorder caused by a deficiency of glycoprotein Ib on the platelet membrane. This glycoprotein is essential for normal platelet function. As a result, individuals with Bernard-Soulier syndrome experience a pronounced tendency to bleed. The platelets in this condition are also unusually large. This contrasts with Glanzmann thrombasthenia, another inherited platelet disorder, which is due to a deficiency of glycoprotein IIb–IIIa. Bleeding disorders can stem from inherited or acquired defects in clotting or platelet function, leading to persistent bleeding.12

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