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Pulmonary Dysfunction in Transfusion-dependent Patients with Thalassemia Major

Dipartimento di Pediatria, Istituto di Fisiologia Umana I, and Dipartimento di Medicina del Lavoro, Università di Milano, Milan, Italy

Correspondence and requests for reprints should be addressed to Edgardo D'Angelo, M.D., Istituto di Fisiologia Umana I, via Mangiagalli 32, 20133 Milan, Italy. E-mail: edgardo.dangelo{at}unimi.it

Pulmonary function tests were performed on 62 transfusion-dependent patients with thalassemia major, ranging in age from 8 to 33 years, and receiving chelation therapy with desferrioxamine or deferiprone. Percent predicted values for FVC, FEV₁, and PEF were significantly reduced, whereas FEV₁/FVC and maximal expiratory flow at 25% FVC were within normal limits, indicating a restrictive disease. Both FVC and FEV₁ were negatively correlated with transfusional iron burden as indexed by age. Single-breath carbon monoxide transfer factor was reduced, even after correction for low hemoglobin concentration, and was negatively correlated with iron burden and iron overload, as indexed by serum ferritin levels. Owing to low hemoglobin concentration, blood-diffusing capacity was reduced, in spite of increased lung capillary blood volume, which was, however, adequate to normalize blood diffusing capacity when hemoglobin concentration was only partially restored by transfusion. The diffusing capacity of the alveolar–capillary membrane was substantially decreased and negatively correlated with age and serum ferritin, the fall being primarily attributed to increased membrane thickness. These findings suggest that lung fibrosis and/or interstitial edema related to iron overload are the main cause of pulmonary dysfunction observed in patients with thalassemia major.

Key Words: alveolar–capillary membrane • iron overload • lung mechanics • pulmonary capillary blood volume • pulmonary diffusing capacity

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