Objective:

Lung involvement is one of known complications of thalassemia. The aim of our study was to determine predominant type of pulmonary dysfunction and its relationship to iron overload in ß thalassemia major patients, at the same time to investigate the presence of the bronchial hyperreactivity.

Material and Methods:

Twenty-seven patients, 15 (56%) girls and 12 (44%) boys, with ß thalassemia major who were followed up from pediatric hematology department were recruited in this study. The patients who were followed up at least for 2 years with ß thalasemia major, having repeated transfusion and chelation therapy, older than 6 years old and without history or signs of chronic lung disease have been included in this study. Pulmonary function test and metacholine bronchial provocation test have been carried out by the same team of technicians for all subjects.

Results:

The mean age of the patients were 11.3±3.81 years. The age of diagnosis was 1.82±2.1 years. Splenectomy was performed in 8 (29.6%) patients. Pulmonary function tests were performed before transfusion and all parameters were normal in all of the patients. Transfusion duration, age, cummulative transfusion volüme, ferritin levels, hemoglobin levels, presence of splenectomy and the presence of hepatomegaly were compared with pulmonary function tests. There was no significant relation between all these parameters and forced vital capacity (FVC), peak expiratory flow (PEF), forced expiratory volume in one second (FEV1), forced expiratory volume in 1 second/ forced vital capacity (FEV1/FVC) and forced expiratory flow at 50% (FEF50) (p>0.05) except age which was correlated inversely with FVC (p=0.008). Forced vital capacity was also inversely correlated with weight and height of the patients (p=0.02, p=0.01). None of patients had bronchial hyperreactivity.

Conclusion:

Restrictive lung disease was the predominant abnormality in multi-transfused thalassemics found in our study. Our data suggest the need to include periodic lung function testing in follow up of all ß thalassemia patients. More studies are necessary to verify the reasons for these respiratory alterations.

Keywords: Restrictive, ß thalassemia major