Hereditary abnormality of platelet aggregation attributable to nucleotide storage pool deficiency.

作者: CM Ingerman , JB Smith , S Shapiro , A Sedar , MJ Silver

DOI: 10.1182/BLOOD.V52.2.332.BLOODJOURNAL522332

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摘要: An abnormality of platelet aggregation has been detected in six family members with mild bleeding tendencies. In citrated platelet-rich plasma, primary induced by ADP or epinephrine and agglutination response to ristocetin were present but second wave collagen suspension absent greatly reduced. Sodium arachidonate-induced was normal although prostaglandin G2 reduced depended entirely on the presence plasma ADP. Further tests indicated that platelets produced prostaglandins did not release ATP thrombin sodium arachidonate. Platelets from patients found contain amounts 5-hydroxytryptamine be unable retain radioactivity during prolonged incubation at 37 degree C radiolabeled 5-hydroxytryptamine. Although electron microscopy revealed an absence very dense bodies, appeared otherwise normal. The findings are discussed relation previous studies nucleotide storage pool deficiency light they shed physiology general.

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