Congenital platelet function defects are problems with platelets, one of the blood elements needed for normal blood clotting. Congenital means present from birth.

Platelet storage pool disorder; Glanzmann's thrombasthenia; Bernard-Soulier syndrome; Platelet function defects - congenital

Platelets are elements in the blood that help form clots. Congenital platelet function defects are bleeding disorders that cause reduced platelet function, even though there are normal platelet counts.

People with these disorders usually have a family history of a bleeding disorder that causes prolonged bleeding after minor cuts or surgery, or easy bruising.

Bernard-Soulier syndrome occurs when platelets lack a substance that sticks to the walls of blood vessels. This disorder may cause severe bleeding.

Glanzmann's thrombasthenia is a condition caused by the lack of a protein required for platelets to clump together. This disorder may also cause severe bleeding.

Platelet storage pool disorder is a mild defect that causes bruising. It is caused by faulty storage of substances inside platelets. These substances are usually released to help platelets function properly.

Images

Blood clot formation

Blood clots

Next Section

Congenital platelet function defects Symptoms

Review Date: 03/02/2009
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)