β-Thalassaemia and sickle cell anaemia as paradigms of hypercoagulability

Kenneth I. Ataga, Maria D. Cappellini, Eliezer A. Rachmilewitz

Research output: Contribution to journalArticle

121 Citations (Scopus)

Abstract

Thalassaemia and sickle cell disease (SCD) represent the most common forms of hereditary haemolytic anaemia and result from a partial or complete lack of synthesis of one of the major α- or β-globin chains of haemoglobin A or from a single amino acid mutation (β6Glu→Val) of the β-globin chain respectively. Although they have different pathophysiologies, patients with these conditions manifest both biochemical and clinical evidence of hypercoagulability. While the frequency of various thrombotic complications may vary in β-thalassaemia and homozygous SCD [sickle cell anaemia (SCA)], patients with both diseases manifest decreased levels of natural anticoagulant proteins, as well as increased markers of thrombin generation and platelet activation. The abnormal phospholipid membrane assymetry present in the red blood cells of β-thalassaemia and SCA patients, with resultant phosphatidylserine exposure appears to play a significant role in the aetiology of the observed hypercoagulable state. This review presents the available data on the aetiology and clinical manifestations of the coagulation and platelet activation that exist in both β-thalassaemia and SCA, as well as the potential therapeutic implications resulting from this hypercoagulability.

Original languageEnglish
Pages (from-to)3-13
Number of pages11
JournalBritish Journal of Haematology
Volume139
Issue number1
DOIs
Publication statusPublished - Oct 2007

Fingerprint

Thalassemia
Thrombophilia
Sickle Cell Anemia
Globins
Platelet Activation
Congenital Hemolytic Anemia
Hemoglobin A
Phosphatidylserines
Thrombin
Anticoagulants
Phospholipids
Erythrocytes
Amino Acids
Mutation
Membranes
Proteins

Keywords

  • β-thalassaemia
  • Nitric oxide
  • Platelet activation
  • Sickle cell anaemia
  • Thrombin generation

ASJC Scopus subject areas

  • Hematology

Cite this

β-Thalassaemia and sickle cell anaemia as paradigms of hypercoagulability. / Ataga, Kenneth I.; Cappellini, Maria D.; Rachmilewitz, Eliezer A.

In: British Journal of Haematology, Vol. 139, No. 1, 10.2007, p. 3-13.

Research output: Contribution to journalArticle

Ataga, Kenneth I. ; Cappellini, Maria D. ; Rachmilewitz, Eliezer A. / β-Thalassaemia and sickle cell anaemia as paradigms of hypercoagulability. In: British Journal of Haematology. 2007 ; Vol. 139, No. 1. pp. 3-13.
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