Oozing and Bruising Due to Abnormal Platelet Function (Thrombocytopathia)

This report describes 6 unrelated patients with mild bleeding disorders caused by abnormalities of platelet function. This syndrome has its onset in childhood and has one or more of these characteristics: easy bruising, spontaneous bleeding from mucosal membranes, excessive bleeding after minor or major surgery, and menorrhagia. Coagulation tests in these patients showed a prolonged bleeding time and a delayed and diminished platelet aggregation response to epinephrine and collagen. The platelet count, platelet adhesiveness, platelet factor-3 release, and factor VIII activity were usually normal. Pedigree studies suggested an autosomal-dominant mode of inheritance. An experimental trial of prednisone therapy in five patients resulted in significant improvement in the bleeding time, yet platelet aggregation test results were unaltered.