Alternate Names : Platelet storage pool disorder, Glanzmann’s thrombasthenia, Bernard-Soulier syndrome, Platelet function defects – congenital
Definition
Congenital platelet function defects are problems with platelets, one of the blood elements needed for normal blood clotting. Congenital means present from birth.
Overview, Causes, & Risk Factors
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.
Pictures & Images
Blood clot formation
Blood clotting normally occurs when there is damage to a blood vessel. Platelets immediately begin to adhere to the cut edges of the vessel and release chemicals to attract even more platelets. A platelet plug is formed, and the external bleeding stops.
Next, small molecules, called clotting factors, cause strands of blood-borne materials, called fibrin, to stick together and seal the inside of the wound. Eventually, the cut blood vessel heals and the blood clot dissolves after a few days.
Blood clots
Blood clots (fibrin clots) are the clumps that result when blood coagulates.
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Congenital platelet function defects : Overview, Causes, & Risk Factors
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Congenital platelet function defects : Symptoms & Signs, Diagnosis & Tests
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Congenital platelet function defects : Treatment
Review Date : 3/2/2009
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.
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