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Sticky platelet syndrome and thrombocythemia.
Eugene P Frenkel1, Eberhard F Mammen
1Department of Medicine, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, TX 75390-8852, USA. Eugene.frenkel@UTSouthwestern.edu
Hematology/Oncology Clinics of North America
|March 12, 2003
Summary
Sticky Platelet Syndrome is an inherited disorder causing hyperactive platelets, leading to arterial clots. Low-dose aspirin effectively treats both the symptoms and platelet hyperactivity.
Area of Science:
- Hematology
- Vascular Biology
- Genetics
Background:
- Platelets play a key role in arterial thrombosis, often linked to endothelial injury.
- Some arterial thromboses arise from "hyperactive" platelets, independent of endothelial damage.
- Hyperaggregable platelets are observed in various acquired conditions, but causality is often unclear.
Purpose of the Study:
- To describe the congenital sticky platelet syndrome (SPS).
- To outline the clinical presentation and genetic basis of SPS.
- To evaluate the efficacy of low-dose aspirin in managing SPS.
Main Methods:
- Characterization of platelet hyperaggregability in response to adenosine diphosphate (ADP) and epinephrine.
- Clinical assessment of patients presenting with arterial vascular occlusions.
- Evaluation of treatment response to low-dose aspirin (81 mg/day).
Main Results:
- Sticky Platelet Syndrome is an autosomal dominant disorder.
- Platelet hyperaggregability in SPS is triggered by ADP, epinephrine, or both.
- Patients experience transient or permanent arterial occlusions, often stress-precipitated.
- Low-dose aspirin therapy reversed clinical symptoms and laboratory hyperaggregability.
Conclusions:
- Sticky Platelet Syndrome is a distinct inherited disorder of platelet hyperreactivity.
- Arterial vascular occlusive events are the primary clinical manifestation.
- Low-dose aspirin is an effective treatment for both the clinical and laboratory features of SPS.