Pulmonary function in thalassaemia major and its correlation with body iron stores

Abstract

This study compared pulmonary function tests (PFTs) with cardiac, pancreatic and liver iron in 76 thalassemia major (TM) patients. Restrictive lung disease was observed in 16%, hyperinflation in 32% and abnormal diffusing capacity in 3%. While no patients met Global Initiative for Chronic Lung Disease criteria for airways obstruction, there were indicators of small airways disease and air trapping. PFTs did not correlate with somatic iron burden, blood counts or haemolysis. Restrictive lung disease was associated with inflammation. We conclude that TM patients have pulmonary abnormalities consistent with small airways obstruction. Restrictive disease and impaired diffusion are less common.

Figure 1

The percentage of patients with abnormal pulmonary function tests. FVC, forced vital capacity; FEV1, forced expiratory volume in 1 second; FEF2575, 25%–75% forced expiratory flow; VC, vital capacity; TLC, total lung capacity; RV, residual volume; MVV, maximum voluntary ventilation; Phase III-N2^, phase III of the single breath nitrogen washout curve; Adj DLCO/VA, diffusing capacity of the lung for carbon monoxide corrected for alveolar volume