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.
