alpha granule deficiency syndrome

alpha granule deficiency syndrome

A rare autosomal dominant bleeding disorder in which the alpha granules of platelets lack adenosine diphosphate, which is critical for proper platelet targeting and clumping in response to injury. The platelet count may be abnormally decreased, and the bleeding time is usually prolonged. The abnormality in platelet granulation is visible with an electron microscope. The most common clinical evidence of the disease is easy bruising in response to injury. Synonym: gray platelet syndrome
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