Platelet Adenine Nucleotide "Storage Pool Deficiency" In Thrombocytopenic Absent Radii Syndrome

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Abstract
To the Editor.— A hemorrhagic diathesis has been described in patients with a platelet functional defect characterized by prolonged bleeding time, normal platelet counts, and abnormal platelet aggregation. The latter is characterized by a poor or absent aggregation with low concentrations of collagen and absence of secondary aggregation with adenosine diphosphate (ADP) and adrenaline.1 Most of these patients are devoid of other defects and the bleeding disorder has been classified as thrombopathia or thrombocytopathia, terms that are confusing and inaccurate. Similar platelet abnormalities have been found in albinos,2,3 in one case of Wiskott-Aldrich syndrome,4 in the Hermansky-Pudlak syndrome,5 and in some cases of connective tissue disease.6 Some of these patients have a deficient storage pool of adenine nucleotides and serotonin, and the abnormality has been referred to as "storage pool deficiency" (SPD).1 This deficiency may be more common than realized, as is evidenced by

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