Congenital platelet function defects

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Blood clot formation

Congenital platelet function defects

Definition:

Congenital (present at birth) platelet function defects are disorders of the platelets, the blood cells essential for the coagulation of the blood.

Alternative Names:
Storage pool disease; Glanzmann's thrombasthenia; Bernard-Soulier syndrome; Platelet function defects - congenital
Causes, incidence, and risk factors:

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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, which may be severe.

Storage pool disease is a mild bleeding disorder that causes bruising. The risk factor is a family history of a bleeding disorder.