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Pulmonary function test in transfusion-dependent β-thalassemia major patients: a pilot study
Soheila Alyasin1, Mozhgan Moghtaderi, Reza Amin
1Department and Laboratory of Pediatric Immunology and Allergy, Shiraz University of Medical Sciences, Shiraz, Iran.
Insights
In children with thalassemia, small airway disease is the most common lung issue, affecting 50% of patients. This study found no link between lung problems and iron overload in these children.
Area of Science:
- Pediatric Pulmonology
- Hematology
- Respiratory Medicine
Background:
- Thalassemia, a genetic blood disorder, can lead to lung complications.
- Iron overload is a common issue in thalassemia patients due to regular blood transfusions.
Purpose of the Study:
- To identify the predominant type of pulmonary dysfunction in children with beta-thalassemia.
- To investigate the relationship between pulmonary dysfunction and iron overload in this population.
Main Methods:
- Pulmonary function tests (spirometry) were conducted on 50 children with thalassemia major.
- Serum ferritin levels were measured to assess iron overload.
- Data on body mass index, hematocrit, and transfusion history were collected.
Main Results:
- 70% of patients exhibited abnormal spirometry results.
- Small airway disease was the most prevalent abnormality, detected in 50% of subjects.
- No significant correlation was found between abnormal pulmonary function and serum ferritin levels.
Conclusions:
- Small airway disease is the predominant pulmonary abnormality in children with thalassemia major.
- Further research is needed to understand the mechanisms and confirm these findings.
Abstract:
Lung involvement is one of known complications of thalassemia. The aim of this study was to determine predominant type of pulmonary dysfunction and its relationship to iron overload in β-thalassemia children. Fifty thalassemia major children with treatment of regular blood transfusion and desferrioxamine participated in the study. Thirty-three boys and 17 girls (median age 12.5 years) with β-thalassemia enrolled in the study. Other information including body mass index, hematocrit, and the number of years of blood transfusion were recorded. Serum ferritin level and hematocrit were 3346 ± 1667 mg/dL and 27.7 ± 2, respectively. Pulmonary function tests were performed in all subjects for detecting pulmonary dysfunction. Thirty-five patients (70%) with thalassemia had abnormal result of spirometry. Obstructive airway disease based on reduced forced expiratory volume in 1 second (FEV(1)) and FEV(1)/forced vital capacity (FVC) ratio <80% was detected in 4 patients (8%). Six patients (12%) showed restrictive pattern, as defined by a reduction FVC <80% and FEV(1)/FVC ratio ≥80%. In this study, small airway involvement based on presence of forced expiratory flow (FEF(25%-75%)) <60%, FEV(1)/FVC ratio >70%, and FVC >80% was detected in 25 subjects (50%). Decreased values of peak expiratory flow rate (PEF) were detected in 23 (46%) and low FEV(1) in 10 (20%) subjects. There was no significant correlation between abnormal pulmonary function test and serum ferritin level in children with thalassemia. This study showed small airway disease was predominant abnormality in thalassemia patients, although additional larger studies are needed to evaluate underlying mechanisms and validate these findings.
