Thalassemia and hypercoagulability

Although the management of β-thalassemia has improved significantly, patients still suffer from many complications, including thrombotic events. A hypercoagulable state has been demonstrated in these conditions, particularly in non-transfusion-dependent β-thalassemia, because of disease-specific contributors that play a role in the pathogenesis, including reactive oxygen species, pathological erythroid cells, circulating microparticles, free heme, and endothelial activation. Splenectomy further contributes to the complexity of thrombotic risk in such patients, together with emerging complications related to increased survival, such as atrial fibrillation. Moreover, in recent years the role of new drugs in further modifying the thrombotic risk of these patients has been demonstrated, as in the case of luspatercept. However, its role still needs to be elucidated. The currently available prevention and clinical management of thrombosis in thalassemia patients mainly relies on the international guidelines for the general population, although, given the peculiar pathophysiology and the disease-related risk factors, robust data and evidence are necessary to develop dedicated guidelines.