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Pulmonary Dysfunction in Transfusion-Dependent Thalassemia and Response to Intensive Chelation Therapy
Neha Panwar1, Sunil Gomber2, Pooja Dewan2
1Department of Pediatrics, University College of Medical Sciences, Delhi. Correspondence to: Dr Neha Panwar, Senior Resident, Department of Pediatrics, University College of Medical Sciences, Delhi 110 095. panwarneha64@gmail.com.
Insights
Pulmonary dysfunction is common in children with thalassemia. Intensive chelation therapy with desferrioxamine (DFO) showed significant improvement in lung function tests, highlighting the importance of screening.
Area of Science:
- Pediatric Hematology
- Pulmonology
- Medical Imaging
Background:
- Transfusion-dependent thalassemia is a chronic condition requiring regular blood transfusions.
- Iron overload is a major complication of thalassemia, affecting multiple organs, including the lungs.
- Pulmonary complications can significantly impact the quality of life and prognosis in children with thalassemia.
Purpose of the Study:
- To assess pulmonary function in children with transfusion-dependent thalassemia.
- To evaluate the potential for reversing lung dysfunction using intensive intravenous chelation with desferrioxamine (DFO).
Main Methods:
- A descriptive study involving 77 children with transfusion-dependent thalassemia.
- Pulmonary function tests (PFTs), including spirometry, total lung capacity (TLC), and diffusion capacity for carbon monoxide (DLCO), were performed.
- Iron load was assessed using serum ferritin (SF) and T2* MRI of the heart and liver. Follow-up PFTs were conducted on 13 children receiving intensive DFO therapy.
Main Results:
- 68.8% of patients exhibited lung dysfunction, primarily diffusional impairment (96%) and reduced TLC (22%).
- Pulmonary function and T2* MRI values showed an inverse correlation with serum ferritin levels.
- Intensive DFO treatment for 4 weeks led to significant improvements in FEV1/FVC ratio, DLCO, and DSF.
Conclusions:
- Pulmonary dysfunction is a frequent comorbidity in multi-transfused children with thalassemia.
- Routine pulmonary function screening should be integrated into the management guidelines for these patients.
- Intravenous desferrioxamine (DFO) shows promise in improving lung function in affected children.
Objective:
To evaluate pulmonary functions in children with transfusion-dependent thalassemia, and its reversal (lung dysfunction) using intensive intravenous chelation with desferrioxamine (DFO) (4 weeks).
Methods:
This descriptive study enrolled 77 children with transfusion-dependent thalassemia. Pulmonary function test (PFT) and iron load (serum ferritin (SF) and T2* MRI of heart and liver) were done. PFT included spirometry, total lung capacity (TLC) by helium dilution test and diffusion capacity by carbon monoxide (DLCO). Follow-up PFT was available for 13 children with moderate to severe lung dysfunction given intravenous DFO.
Results:
50 (68.8%) patients had lung dysfunction, most commonly diffusional impairment (48; 96%), and reduced TLC (11; 22%); and none had obstructive pattern. 9 (81.8%) patients with restrictive defect had moderate to severely deranged DLCO. PFT and T2* MRI values were inversely correlated with serum ferritin. Among 13 patients receiving intensive chelation for 4 weeks, significant improvement was noticed in forced expiratory volume in one minute/ forced vital capacity ratio (DFEV1/FVC) (P=0.009), DDLCO (P=0.006) and DSF (P=0.01).
Conclusions:
Pulmonary dysfunction is common in children with multi-transfused thalassemia, and routine screening by PFT needs to be part of the management guidelines.
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