STUDIES ON THE HEMOSTATIC DEFECT IN A COMPLICATED SYNDROME - AN INVERSE SCOTT SYNDROME PLATELET MEMBRANE ABNORMALITY

The Stormorken syndrome is a multifacetted syndrome including a bleedi ng tendency. No deviations were found in the coagulation- or fibrinoly tic systems. Platelet number was low normal, and size abnormal, wherea s EM findings were unremarkable. Survival time was half normal. Clot r etraction was initially rapid, but clearly decreased, whereas prothrom bin consumption was also initially rapid, but complete. Membrane GP's were normal, so was AA metabolism, PI-cycle, gran ule storage and secr etion, and c-AMP function, whereas 5-HT uptake and storage was decreas ed. Optical platelet aggregation was low normal with all physiological agonists. The only clearly abnormal finding was that coagulant activi ty was present on non stimulated platelets at the same level as kaolin -stimulated normal platelets. This indicated a platelet abnormality wh ich should lead to a thrombogenic, not to a haemorrhagic trait. This p aradox may have its origin in rheology, because when challenged with i n vivo shear rates in an ex vivo perfusion chamber, platelet cohesion was abnormally low. Further studies to better delineate the membrane a bnormality are underway.