Last update:
06-Feb-2008

The thalassemias are a group of genetically transmittable diseases characterized by the reduction in synthesis of one or numerous hemoglobin chains. During recent years, the prognosis of the disease has improved dramatically due to new therapeutic procedures and the median age of survival approaches almost normal limits. However, the disease is complicated by significant clinical problems such as heart failure, structural damage to the arteries and thromboembolic episodes, the pathogenesis of which is not yet clearly understood. It is believed that many factors play a crucial role in the etiology of cardiovascular disorders such as elevated iron levels, adhesion molecules, including cytokines, acute phase proteins and coagulation factors, as well as the vascular endothelial cells, red blood cells and platelets. In this review, analysis is made of the role of all these factors, targeting mostly the endothelial cells, in order to provide a better idea of the pathogenesis mechanisms of the thalassemias and their complications which could lead to more successful therapeutic management.

Key words: Beta-thalassemia, Cardiovascular disease.