Vascular and hemostatic alterations associated with pulmonary hypertension in beta-thalassemia hemoglobin E patients receiving regular transfusion and iron chelation

摘要

Introduction: Pulmonary hypertension (PH) is the commonest cardiac complication in beta-thalassemia intermedia, including beta-thalassemia/hemoglobin E (beta-thal/HbE), and is strongly associated with splenectomy. We aimed to define the prevalence and comprehensively explore mechanisms of PH in beta-thal/HbE patients receiving regular transfusion and iron chelation, which were reported to alleviate PH.