Hypercoagulability in sickle cell disease and beta-thalassemia

Sylvia T. Singer, Kenneth Ataga

Research output: Contribution to journalReview article

13 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)639-645
Number of pages7
JournalCurrent Molecular Medicine
Volume8
Issue number7
DOIs
StatePublished - Dec 1 2008
Externally publishedYes

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Thrombophilia
beta-Thalassemia
Sickle Cell Anemia
Anticoagulants
Hemoglobinopathies
Thalassemia
Platelet Aggregation Inhibitors
Platelet Activation
Hemostatics
Hemolysis
Reperfusion Injury
Phospholipids
Nitric Oxide
Erythrocytes
Chemical activation
Cell Membrane
Platelets
Coagulation
Proteins
Blood

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Molecular Medicine
  • Molecular Biology

Cite this

Hypercoagulability in sickle cell disease and beta-thalassemia. / Singer, Sylvia T.; Ataga, Kenneth.

In: Current Molecular Medicine, Vol. 8, No. 7, 01.12.2008, p. 639-645.

Research output: Contribution to journalReview article

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