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

Abstract:

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

journal_name

Blood

journal_title

Blood

authors

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

subject

Has Abstract

pub_date

1978-08-01 00:00:00

pages

332-44

issue

2

eissn

0006-4971

issn

1528-0020

journal_volume

52

pub_type

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