Thalassemia and hypercoagulability

SummaryThalassemia 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.