"Impotent" platelets in albinos with prolonged bleeding times.

作者: Harold M. Maurer , James A. Wolff , Sue Buckingham , Arthur R. Spielvogel

DOI: 10.1182/BLOOD.V39.4.490.490

关键词:

摘要: Functional, biochemical, and morphologic platelet abnormalities are reported in four children with the syndrome of albinism, mild bleeding tendency, prolonged time, normal count. In these children, primary aggregation adenosine diphosphate occurred normally, but secondary was impaired. Collagen norepinephrine produced almost no aggregation. Platelet content serotonin (5-HT) markedly reduced, uptake retention 5-HT by platelets vivo vitro poor. one child who given a tryptophan load, urinary metabolites were normal, suggesting that there evidence block synthetic pathway gastrointestinal tract. Electron microscopy revealed an absence densely osmophilic granules poor platelets. Platelets from other albinos history contained amounts granules.

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