Thalassemia is a congenital hemolytic disease caused by defective globin synthesis resulting in decreased quantity of globin chains. Although the life expectancy of β-thalassemia patients has markedly improved over the last few years, patients still suffer from many complications of this congenital disease. The presence of a high incidence of thromboembolic events, mainly in β-thalassemia intermedia, has led to the identification of a hypercoagulable state in these patients. In this paper, we review the molecular and cellular mechanisms leading to hypercoagulability in β-thalassemia, with a special focus on thalassemia intermedia being the group with the highest incidence of thrombotic events as compared to other types of thalassemias. We also discuss the recommendations for thrombosis prophylaxis in these patients.

Thalassemia and hypercoagulability / A.T. Taher, Z.K. Otrock, I. Uthman, M.D. Cappellini. - In: BLOOD REVIEWS. - ISSN 0268-960X. - 22:5(2008 Sep), pp. 283-292.

Thalassemia and hypercoagulability

M.D. Cappellini
Ultimo
2008

Abstract

Thalassemia is a congenital hemolytic disease caused by defective globin synthesis resulting in decreased quantity of globin chains. Although the life expectancy of β-thalassemia patients has markedly improved over the last few years, patients still suffer from many complications of this congenital disease. The presence of a high incidence of thromboembolic events, mainly in β-thalassemia intermedia, has led to the identification of a hypercoagulable state in these patients. In this paper, we review the molecular and cellular mechanisms leading to hypercoagulability in β-thalassemia, with a special focus on thalassemia intermedia being the group with the highest incidence of thrombotic events as compared to other types of thalassemias. We also discuss the recommendations for thrombosis prophylaxis in these patients.
Settore MED/09 - Medicina Interna
set-2008
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/46836
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