Medical Definition of Bernard Soulier syndrome
1.
A congenital disorder where the platelets lack receptors which allow them to adhere to the walls of blood vessels. The genetic deficiency in platelet membrane glycoprotein Ib allows platelets to aggregate normally (c.f. Glanzmann's thrombasthenia) but they do not stick to collagen of sub endothelial basement membrane. The net result is a platelet function disorder that causes a prolonged bleeding time.
Symptoms include nosebleeds, easy bruising, heavy menstrual periods, bleeding gums and increased post-operative bleeding. Avoidance of aspirin, anti-inflammatories or any other agents which can cause bleeding is critical.
(06 Oct 1997)
Lexicographical Neighbors of Bernard Soulier Syndrome
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