Relation of ferritin levels to pulmonary function in patients with thalassemia major and the acute effects of transfusion

Abstract

We studied 36 patients (17 males and 19 females) with thalassemia major by performing pulmonary function testing (PFT), arterial blood gas analysis (ABG), as well as determining the serum ferritin level. In addition, 19 of these patients were transfused with two units of packed cells, and a repeat ABG and PFT were performed. Twenty-three patients had normal PFTs, eleven patients (30.6%) showed a restrictive pattern (significant decrease in both TLC and DLCO), and only two patients (5.6%) showed an obstructive pattern. A significant negative correlation was found between serum ferritin and restrictive parameters, DLCO and TLC (p = 0.01 and p = 0.03, respectively). This correlation was even stronger after transfusion. Controlling for age, ferritin was still negatively correlated with DLCO (p = 0.04), but no longer with TLC. There was no correlation between age and DLCO or age and TLC; however, there was a statistically significant negative correlation between age and FVC (p = 0.003). Analysis of patients who were transfused revealed a significant decrease in forced vital capacity (89 +/- 4% vs. 74 +/- 5% of predicted; p-value < 0.001) and in maximum midexpiratory flow rate (79 +/- 4% vs. 67 +/- 5% of predicted; p-value = 0.004). For patients older than 15 yr of age, there was a statistically significant decrease in FEV1/FVC (84 +/- 2 vs. 83 +/- 2%; p-value = 0.04). The ABGs showed no significant change post-transfusion. In this study, PFT findings in thalassemia major were restrictive and correlated with serum ferritin level. Transfusion had an acute effect on the obstructive parameters of PFT.

PULMONARY FUNCTION TEST IN TRANSFUSION-DEPENDENT Β-Thalassemia PATIENTS

“…According to our results, restrictive pattern was the most common finding (72.7%) in PFT, which is similar to other studies [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. As the data show, iron accumulation because of repeated transfusion has been proposed as the likely mechanism that causes pulmonary dysfunction in thalassemic patients [5,8,11,13,15,17].…”

“…As the data show, iron accumulation because of repeated transfusion has been proposed as the likely mechanism that causes pulmonary dysfunction in thalassemic patients [5,8,11,13,15,17]. In the study by Parakh and colleagues for finding the pathogenesis of respiratory impairment in thalassaemia patients, PFT and carbon monoxide diffusion capacity (DLco) were done for 31 patients over 8 years.…”

“…In evaluation of pulmonary system, most reports have shown that restrictive pattern is the most frequent abnormal pattern in the pulmonary function test (PFT) in thalassemia patients [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19], but there is no general agreement about the physiologic basis of this problem [7,9,14,18,19]. Fisola and colleagues [15] have shown that restrictive pattern in elder thalassemic patients is associated with their serum ferritin.…”

“…But this treatment has some complications, such as progressive iron deposition in vital organs, which impairs their functions [3]. There are some debates about abnormalities of respiratory function and causes of its dysfunction [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22].…”

Pulmonary dysfunction in children with beta thalassemia major in relation with iron overload - a cross sectional hospital based study

“…This is similar to other published studies done by Rahim et al 4 and Bourli et al 6 where restrictive abnormalities were most frequently reported. Studies done by Kanj et al 4 and Amin et al 7 showed both Obstructive and Restrictive pattern of Respiratory dysfunction.…”