Pulmonary hypertension, interstitial lung fibrosis, and lung iron deposition in thalassaemia major

Although restrictive lung disease is the predominant abnormality of pulmona ry function in patients with thalassaemia major (TM), its aetiology and its association with pulmonary hypertension (PH) detected in some patients wit h TM remains unknown. We report a patient with TM, iron overload, frequent pulmonary infections, and progressive severe precapillary PH over the previ ous 5 years. A severe restrictive pattern and interstitial lung fibrosis we re revealed by pulmonary function tests and high resolution computed tomogr aphy, respectively. This presentation suggests that interstitial fibrosis m ay complicate lung involvement in TM and can significantly contribute to th e development of PH.