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THE PLATELET DEFECT ASSOCIATED WITH ALBINISM

In 1967, Hardisty and Hutton’ described a series of patients with mild bleeding tendencies whose platelets failed to aggregate on exposure to collagen and gave no second phase of aggregation on exposure to adenosine diphosphate ( A D P ) , epinephrine, or thrombin. They suggested that the bleeding tendency of these patients, among whom were two unrelated women with total albinism, might reflect a defect of the release of A D P from their platelets. We present here the results of further work that confirms this suggestion and offers some explanation of the underlying mechanisms involved.