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Thromb Haemost 1995; 74(05): 1244-1251
DOI: 10.1055/s-0038-1649920
Original Article
Clinical Studies
Schattauer GmbH Stuttgart

Studies on the Haemostatic Defect in a Complicated Syndrome

An Inverse Scott Syndrome Platelet Membrane Abnormality?
H Stormorken
1   The Research Institute for Internal Medicine, Oslo, Norway
3   The Nycomed Pharma AS, Oslo, Norway
,
H Holmsen
2   The Dept of Biochemistry and Molecular Biology, University of Bergen, Oslo, Norway
,
R Sund
2   The Dept of Biochemistry and Molecular Biology, University of Bergen, Oslo, Norway
,
K S Sakariassen
3   The Nycomed Pharma AS, Oslo, Norway
,
T Hovig
4   The Electronmicroscopic Laboratory, Dept, of Pathology, University Hospital, Rikshospitalet, Oslo, Norway
,
E Jellum
5   The Institute of Clinical Biochemistry, University Hospital, Rikshospitalet, Oslo, Norway
,
N O Solum
1   The Research Institute for Internal Medicine, Oslo, Norway
› Author Affiliations
Further Information

Publication History

Received 10 April 1995

Accepted after resubmission 07 August 1995

Publication Date:
27 July 2018 (online)

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Summary

The Stormorken syndrome is a multifacetted syndrome including a bleeding tendency. No deviations were found in the coagulation- or fibrinolytic systems. Platelet number was low normal, and size abnormal, whereas EM findings were unremarkable. Survival time was half normal. Clot retraction was initially rapid, but clearly decreased, whereas prothrombin consumption was also initially rapid, but complete. Membrane GP’s were normal, so was AA metabolism, PI-cycle, granule storage and secretion, and c-AMP function, whereas 5-HT uptake and storage was decreased. Optical platelet aggregation was low normal with all physiological agonists. The only clearly abnormal finding was that coagulant activity was present on non stimulated platelets at the same level as kaolin-stimulated normal platelets. This indicated a platelet abnormality which should lead to a thrombogenic, not to a haemorrhagic trait. This paradox may have its origin in rheology, because when challenged with in vivo shear rates in an ex vivo perfusion chamber, platelet cohesion was abnormally low. Further studies to better delineate the membrane abnormality are underway.

 

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