TITLE

Ten Years' Experience with the "Sticky Platelet Syndrome"

AUTHOR(S)
Mammen, Eberhard F.
PUB. DATE
January 1995
SOURCE
Clinical & Applied Thrombosis/Hemostasis;Winter1995, Vol. 1 Issue 1, p66
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
We describe a 10-year experience with a congenital platelet abnormality characterized by hyperaggregability with ADP and/or epinephrine. The syndrome has so far been identified in > 200 patients and their families. Clinical symptoms are transient or permanent arterial occlusions, thrombotic in nature affecting especially the coronary and cerebral vasculature including ophthalmic vessels. The patients have no identifiable risk factors and are usually young (5-45 years), and other hypercoagulable states are excluded. In many cases the vascular accident is precipitated by severe stressful events. Occasionally, the syndrome found in patients with recurrent venous thromboembolism while they are on optimal oral anticoagulant therapy. In the laboratory, the patients' platelets are hyperaggregable with decreasing ADP and/or epinephrine concentrations added to platelet-rich plasma. Two forms are identified: type 1 is market by hyperaggregability with ADP and epinephrine, while type II evidences hyperaggregability only with epinephrine. No abnormalities are found when other aggregation stimulators are used. By electron microscopy the platelets are more readily activated by surface contact, with a greater tendency toward aggregate formation. Plasma levels of platelet release proteins, platelet factor 4 and β-thromboglobulin are not elevated. The syndrome is treated with low-dose aspirin, which reverses the hyperaggregability and improves clinical symptoms. We hypothesize that in vivo adrenaline release leads to increased platelet clumping, which can transiently or permanently occlude small vessels. It is conceivable that altered surface receptors on the platelets might be responsible for this congenital problem.
ACCESSION #
16702263

 

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