Congenital Platelet Function Defects
Alternate Names : Bernard-Soulier Syndrome, Glanzmann's Thrombasthenia, Platelet Function Defects - Congenital, Storage Pool Disease
Definition Congenital platelet function defects are disorders of platelet function, the blood cells essential for the coagulation of the blood, that is present at birth.
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Overview, Causes, & Risk Factors
Congenital platelet function defects are bleeding disorders characterized by abnormal platelet function in spite of normal platelet counts. There is usually a family history of a bleeding disorder consisting of prolonged bleeding or easy bruising.
Bernard-Soulier syndrome is a congenital disorder where the platelets lack receptors to adhere to the walls of the blood vessels. Bleeding may be severe with this disorder.
Glanzmann's thrombasthenia is a condition caused by lack of a protein required for platelet aggregation (clumping) This disorder may cause mucosal and postoperative bleeding, and it may also be severe.
Storage pool disease is a mild bleeding disorder that causes bruising. The risk factor is a family history of a bleeding disorder.
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