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

•PH remains prevalent in β-thal/HbE with regular transfusion and iron chelation.•Splenectomy is not associated with PH in regularly transfused β-thal/HbE.•We propose new mechanisms for PH in regularly transfused β-thal/HbE.•Endothelial dysfunction plays a crucial role in β-thal-associated PH.•NO depletion and TF-independent coagulation contribute to β-thal-associated PH.