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Published on: November 5, 2019
Hypercoagulability in sickle cell disease: new approaches to an old problem
1Division of Hematology/Oncology, University of North Carolina at Chapel Hill, CB# 7305, 3009 Old Clinic Bldg, Chapel Hill, NC 27599-7305, USA. kataga@med.unc.edu
Patients with sickle cell disease (SCD) show chronic coagulation and platelet activation, suggesting these may drive disease complications. Further research into hypercoagulability and clinical trials are needed to confirm this role.
Area of Science:
- Hematology
- Vascular Biology
- Thrombosis Research
Background:
- Sickle cell disease (SCD) is characterized by chronic activation of coagulation and platelets, even during steady states.
- Elevated markers of thrombin generation, depleted anticoagulants, and altered fibrinolysis are observed in SCD patients.
- The role of these prothrombotic changes in SCD pathophysiology remains unclear, with debate on whether they are causal or epiphenomenal.
Purpose of the Study:
- To investigate the contribution of hypercoagulability and platelet activation to the pathophysiology of sickle cell disease.
- To explore the potential role of these hemostatic abnormalities in macrovascular thrombotic complications associated with SCD.
- To highlight the need for further research into the pathogenesis of hypercoagulability in SCD.
Main Methods:
- Review of existing evidence on coagulation and platelet activation markers in SCD patients.
- Analysis of the association between prothrombotic states and thrombotic complications in SCD.
- Consideration of soluble CD40 ligand's biological activity in SCD pathophysiology.
Main Results:
- Patients with SCD consistently display markers of increased thrombin generation and chronic platelet activation.
- Evidence suggests that these prothrombotic states may contribute to macrovascular thrombotic events in SCD.
- The precise role of hypercoagulability in SCD pathogenesis requires deeper investigation.
Conclusions:
- Coagulation and platelet activation are significant features of sickle cell disease, potentially contributing to its complications.
- Understanding the pathogenesis of hypercoagulability is crucial for defining its role in SCD.
- Well-controlled clinical trials with anticoagulants and antiplatelet agents are warranted to evaluate therapeutic strategies.
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