What question did this study set out to answer?

Characterize the physiological and biochemical mechanisms causing defective platelet aggregation in a family presenting with mild bleeding tendencies.

August 1, 1978Blood

Hereditary abnormality of platelet aggregation attributable to nucleotide storage pool deficiency

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Authors

CICM IngermanThomas Jefferson University JSJ. Bryan SmithTH Köln - University of Applied Sciences SSSandor S. ShapiroThomas Jefferson University

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Implication

Familial investigation reveals defective secondary platelet aggregation and dense granule loss in six related individuals, indicating hereditary storage pool deficiency.

Key Points

Characterize the physiological and biochemical mechanisms causing defective platelet aggregation in a family presenting with mild bleeding tendencies.
Assessed platelet aggregation and agglutination in citrated platelet-rich plasma from six affected family members using agonists including ADP, epinephrine, ristocetin, collagen, arachidonate, and prostaglandin G2.
Measured prostaglandin generation, ATP release following thrombin and arachidonate stimulation, intracellular ADP and 5-hydroxytryptamine content, radiolabeled serotonin retention, and platelet ultrastructure via electron microscopy.
Platelets exhibited normal primary aggregation to ADP or epinephrine and normal arachidonate-induced aggregation, but lacked second-wave aggregation and showed markedly reduced aggregation to collagen and prostaglandin G2.
Platelets produced prostaglandins normally but failed to release ATP in response to thrombin, harbored reduced levels of ADP and 5-hydroxytryptamine, and exhibited an absence of dense bodies on electron microscopy.

Cite This Study

Ingerman et al. (1978) studied this question.

synapsesocial.com/papers/6a9bf184182ce59ecd179441 https://doi.org/10.1182/blood.v52.2.332.bloodjournal522332

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Implication

Familial investigation reveals defective secondary platelet aggregation and dense granule loss in six related individuals, indicating hereditary storage pool deficiency.

Hereditary abnormality of platelet aggregation attributable to nucleotide storage pool deficiency

Discussion

Member takes

Implication

Key Points

Cite This Study

Hereditary abnormality of platelet aggregation attributable to nucleotide storage pool deficiency

Discussion

Member takes

Implication

Key Points

Cite This Study

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