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Hypercoagulability in sickle cell disease: a curious paradox.
Kenneth I Ataga1, Eugene P Orringer
1Division of Hematology/Oncology, Department of Medicine, University of North Carolina Comprehensive Sickle Cell Program, Chapel Hill, North Carolina 27599-7305, USA. kataga@med.unc.edu
The American Journal of Medicine
|December 25, 2003
Summary
Sickle cell disease involves increased blood clotting and platelet activation. Targeting these pathways may offer new treatments for sickle cell disease complications.
Area of Science:
- Hematology
- Vascular Biology
- Thrombosis
Background:
- Sickle cell disease (SCD) is characterized by abnormal red blood cells, leading to vaso-occlusion and organ damage.
- Evidence suggests significant activation of blood coagulation and platelets in SCD patients during both steady state and painful crises.
- This hypercoagulable state contributes to the complex pathophysiology of SCD.
Purpose of the Study:
- To review the evidence for hypercoagulability and platelet activation in various forms of sickle cell disease.
- To discuss the potential therapeutic implications of targeting coagulation and platelet pathways in SCD management.
Main Methods:
- Review of existing literature on coagulation and platelet markers in sickle cell disease.
- Analysis of studies examining thrombin generation, anticoagulant protein levels, and fibrinolytic activity.
- Assessment of platelet activation markers (e.g., CD62P, CD40L, thrombospondin) in SCD.
Main Results:
- Elevated thrombin generation and depleted anticoagulant proteins are observed in SCD.
- The fibrinolytic system shows abnormal activation in sickle cell disease.
- Platelet activation is indicated by increased surface markers and circulating thrombospondin levels.
- Increased thrombin levels impact endothelial cells and blood vessels, contributing to SCD pathophysiology.
Conclusions:
- Hypercoagulability and platelet activation are key features of sickle cell disease.
- Therapeutic strategies aimed at reducing thrombin generation or platelet activation may be beneficial for SCD treatment and complication prevention.
- Further research into these pathways could lead to novel therapeutic approaches for homozygous sickle cell anemia, hemoglobin SC disease, hemoglobin SD disease, and sickle cell-beta-thalassemia.