Abstract
Sickle cell disease (SCD) and beta-thalassemia (also referred to as β-thalassemia) are common hereditary hemoglobinopathies with differing pathophysiologies and clinical courses. However, patients with both diseases exhibit increased platelet and coagulation activation, as well as decreased levels of natural anticoagulant proteins. In addition, they are characterized by thrombotic complications that may share a similar pathogenesis. The pathogenesis of hypercoagulability is likely multifactorial, with contributions from the abnormal red blood cell (RBC) phospholipid membrane asymmetry, ischemia-reperfusion injury, and chronic hemolysis with resultant nitric oxide depletion. More studies are needed to better define the contribution of hemostatic activation to the pathophysiology of SCD and beta-thalassemia. Furthermore, adequately controlled studies using anticoagulants and antiplatelet agents are warranted to define the role of hypercoagulability in specific complications of these diseases.
Keywords: Sickle cell disease, beta-thalassemia, thrombosis, hypercoagulable state, platelet activation
Current Molecular Medicine
Title: Hypercoagulability in Sickle Cell Disease and Beta-Thalassemia
Volume: 8 Issue: 7
Author(s): Sylvia T. Singer and Kenneth I. Ataga
Affiliation:
Keywords: Sickle cell disease, beta-thalassemia, thrombosis, hypercoagulable state, platelet activation
Abstract: Sickle cell disease (SCD) and beta-thalassemia (also referred to as β-thalassemia) are common hereditary hemoglobinopathies with differing pathophysiologies and clinical courses. However, patients with both diseases exhibit increased platelet and coagulation activation, as well as decreased levels of natural anticoagulant proteins. In addition, they are characterized by thrombotic complications that may share a similar pathogenesis. The pathogenesis of hypercoagulability is likely multifactorial, with contributions from the abnormal red blood cell (RBC) phospholipid membrane asymmetry, ischemia-reperfusion injury, and chronic hemolysis with resultant nitric oxide depletion. More studies are needed to better define the contribution of hemostatic activation to the pathophysiology of SCD and beta-thalassemia. Furthermore, adequately controlled studies using anticoagulants and antiplatelet agents are warranted to define the role of hypercoagulability in specific complications of these diseases.
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Cite this article as:
Singer T. Sylvia and Ataga I. Kenneth, Hypercoagulability in Sickle Cell Disease and Beta-Thalassemia, Current Molecular Medicine 2008; 8 (7) . https://dx.doi.org/10.2174/156652408786241366
DOI https://dx.doi.org/10.2174/156652408786241366 |
Print ISSN 1566-5240 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5666 |
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