Abstract

Thalassemia major (TM) is characterized by abnormal hemoglobin synthesis, which results in decreased oxygen delivery to the tissues, ineffective erythropoiesis and iron overload. The purpose of this study was to find out the predominant type of lung mechanical abnormalities in TM patients, the prevalence of the change in pulmonary diffusing capacity and to search the association of cytokines with pulmonary function tests (PFTs). TM patients (n = 26) who were on regular transfusion program were included in the study. Pretransfusional and posttransfusional PFTs were performed. Before transfusion, blood samples were collected for complete blood count, serum ferritin, zinc, transforming growth factor-β1 (TGF-β1), interleukin (IL)-8 and IL-10 levels. Obstructive lung pattern was the predominant pulmonary sequela. Male gender was the main risk factor for pulmonary function abnormality. Because of very low values, serum IL-10 levels of all patients were undetectable, and serum IL-8 levels could be measured only in six patients. Median serum IL-8 level was higher in patients with abnormal PFT. Pretransfusional single breath diffusion of carbon monoxide (DLCO ) correlated negatively with serum TGF-β1 and ferritin levels. After transfusion, there was a statistically significant decrease in forced expiratory volume in 1 s, forced expiratory flow between 25% and 75% of vital capacity, and airway conductance but significant increase in DLCO . Although they are mostly asymptomatic, TM patients have important PFT abnormalities. Blood transfusion may have an acute deleterious effect on pulmonary functions. The disturbed pulmonary functions were found to correlate with IL-8 and TGF-β levels. Relations of different cytokines with different PFT parameters suggest that the immune system is effective pulmonary dysfunction.

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