The relationship between increased iron load and respiratory function tests in patients diagnosed with transfusion dependent thalassemia
Abstract
Aims: Thalassaemia syndromes are the most common single gene disorders affecting more than 200 million people worldwide. Beta thalassaemia (BT) is the most common cause of transfusion-dependent thalassaemia (TDT). It has been reported in studies that iron accumulation occurs in the lungs, especially in the alveolo-capillary membrane, and the frequency of parenchymal disease increases in patients receiving frequent blood transfusions. In our study, we aimed to investigate whether there is a correlation between iron overload and pulmonary function in patients with TDT.
Methods: The study included 61 patients aged between 18 and 45 years with a diagnosis of TDT who were followed up in the hematology clinic of our tertiary care center between 2018 and 2023. Based on spirometry measurements, the pattern of respiratory impairment was defined and correlated with serum ferritin levels.
Results: The mean age of the 61 patients included in the study was 24.83±6.02 years and 33 were female and 28 were male. The mean ferritin value was 3150.88±2553.51 ng/ml. The annual number of transfusions was 15.39±1.90. According to the PFT results, mean FVC % value was 81.59±9.28, mean FEV1 % value was 82.11±7.6, mean FEV1/FVC % value was 102.55±7.63. Mean ferritin values were found to be significantly higher in patients diagnosed with TDT with restrictive lung pattern (p=0.004).
Conclusion: Our study showed that high ferritin levels are related to increased restrictive lung disease in the adult age group.
Keywords
References
- Sohn EY, Kato R, Noetzli LJ, et al. Exercise performance in thalassemia major: correlation with cardiac iron burden. Am J Hematol. 2013;88(3):193-197.
- Origa, R. β-Thalassemia. Genet Med. 2017;19(6):609-619.
- Arora M, Chandra J, Suri JC, Narayan S, Dutta AK. Pulmonary function tests in beta thalassemia. Indian J Pediatr. 2001;68(3):239-242.
- Kanj N, Shamseddine A, Gharzeddine W, et al. Relation of ferritin levels to pulmonary function in patients with thalassemia major and the acute effects of transfusion. Eur J Haematol. 2000;64(6):396-400.
- Kazazian Jr HH. The thalassemia syndromes: molecular basis and prenatal diagnosis in 1990. Semin Hematol. 1990;27(3):209-228.
- Witzleben CL, Wyatt J. The effect of long survival on the pathology of thalassaemia major. J Pathol Bacteriol. 1961;82(1):1-12.
- Factor JM, Pottipatti SR, Rappoport I, Rosner IK, Lesser ML, Giardina PJ. Pulmonary function abnormalities in thalassemia major and the role of iron overload. Am J Respir Crit Care Med. 1994;149(6):1570-1574.
- Bacalo A, Kivity S, Heno N, Greif Z, Greif J, Topilsky M. Blood transfusion and lung function in children with thalassemia major. Chest. 1992;101(2):362-365.
- Piatti G, Allegra L, Ambrosetti U, Cappellini MD, Turati F, Fiorelli G. Beta-thalassemia and pulmonary function. Haematologica. 1999;84(9):804-808.
- Alyasin S, Moghtaderi M, Amin R, Kashef S, Karimi M. Pulmonary function test in transfusion-dependent β-thalassemia major patients: a pilot study. Pediatr Hematol Oncol. 2011;28(4);329-333.
- Cappell DF, Hutchison HE, Jowett M. Transfusional siderosis: the effects of excessive iron deposits on the tissues. J Pathol Bacteriol. 1975;74;245-264.
- Papanikolaou G, Pantopoulos K. Iron metabolism and toxicity. Toxicol App Pharmacol. 2005;202(2):199-211.
- Ooi GC, Khong PL, Lam WK, Trendell-Smith NJ, Tsang KWT. Pulmonary iron overload in thalassemia major presenting as small airway disease. Acta Haematol. 2002;108(1):43-46.
- Bourli E, Dimitriadou M, Economou M, et al. Restrictive pulmonary dysfunction and its predictors in young patients with β‐thalassaemia major. Pediatr Pulmonol. 2012;47(8):801-807.
- Eldor A, Rachmilewitz EA. The hypercoagulable state in thalassemia. Blood. 2002;99(1):36-43.
- Sumiyoshi A, Thakerngpol K, Sonakul D. Pulmonary microthromboemboli in thalassemic cases. Southeast Asian J Trop Med Public Health. 1992;23(Suppl 2):29-31.
- Chatterjee R, Bajoria R. Critical appraisal of growth retardation and pubertal disturbances in thalassemia. Ann New York Acad Sci. 2010;1202(1);100-114.
- Li AM, Chan D, Li CK, Wong E, Chan Y, Fok T. Respiratory function in patients with thalassaemia major: relation with iron overload. Arch Dis Childhood. 2002;87(4):328-330.
- Kanj N, Shamseddine A, Gharzeddine W, et al. Relation of ferritin levels to pulmonary function in patients with thalassemia major and the acute effects of transfusion. Eur J Haematol. 2000;64(6):396-400.
- Chan KC, Au CT, Leung AW, et al. Pulmonary function in patients with transfusion-dependent thalassemia and its associations with iron overload. Scientific Rep. 2023;13(1):3674.
Abstract
References
- Sohn EY, Kato R, Noetzli LJ, et al. Exercise performance in thalassemia major: correlation with cardiac iron burden. Am J Hematol. 2013;88(3):193-197.
- Origa, R. β-Thalassemia. Genet Med. 2017;19(6):609-619.
- Arora M, Chandra J, Suri JC, Narayan S, Dutta AK. Pulmonary function tests in beta thalassemia. Indian J Pediatr. 2001;68(3):239-242.
- Kanj N, Shamseddine A, Gharzeddine W, et al. Relation of ferritin levels to pulmonary function in patients with thalassemia major and the acute effects of transfusion. Eur J Haematol. 2000;64(6):396-400.
- Kazazian Jr HH. The thalassemia syndromes: molecular basis and prenatal diagnosis in 1990. Semin Hematol. 1990;27(3):209-228.
- Witzleben CL, Wyatt J. The effect of long survival on the pathology of thalassaemia major. J Pathol Bacteriol. 1961;82(1):1-12.
- Factor JM, Pottipatti SR, Rappoport I, Rosner IK, Lesser ML, Giardina PJ. Pulmonary function abnormalities in thalassemia major and the role of iron overload. Am J Respir Crit Care Med. 1994;149(6):1570-1574.
- Bacalo A, Kivity S, Heno N, Greif Z, Greif J, Topilsky M. Blood transfusion and lung function in children with thalassemia major. Chest. 1992;101(2):362-365.
- Piatti G, Allegra L, Ambrosetti U, Cappellini MD, Turati F, Fiorelli G. Beta-thalassemia and pulmonary function. Haematologica. 1999;84(9):804-808.
- Alyasin S, Moghtaderi M, Amin R, Kashef S, Karimi M. Pulmonary function test in transfusion-dependent β-thalassemia major patients: a pilot study. Pediatr Hematol Oncol. 2011;28(4);329-333.
- Cappell DF, Hutchison HE, Jowett M. Transfusional siderosis: the effects of excessive iron deposits on the tissues. J Pathol Bacteriol. 1975;74;245-264.
- Papanikolaou G, Pantopoulos K. Iron metabolism and toxicity. Toxicol App Pharmacol. 2005;202(2):199-211.
- Ooi GC, Khong PL, Lam WK, Trendell-Smith NJ, Tsang KWT. Pulmonary iron overload in thalassemia major presenting as small airway disease. Acta Haematol. 2002;108(1):43-46.
- Bourli E, Dimitriadou M, Economou M, et al. Restrictive pulmonary dysfunction and its predictors in young patients with β‐thalassaemia major. Pediatr Pulmonol. 2012;47(8):801-807.
- Eldor A, Rachmilewitz EA. The hypercoagulable state in thalassemia. Blood. 2002;99(1):36-43.
- Sumiyoshi A, Thakerngpol K, Sonakul D. Pulmonary microthromboemboli in thalassemic cases. Southeast Asian J Trop Med Public Health. 1992;23(Suppl 2):29-31.
- Chatterjee R, Bajoria R. Critical appraisal of growth retardation and pubertal disturbances in thalassemia. Ann New York Acad Sci. 2010;1202(1);100-114.
- Li AM, Chan D, Li CK, Wong E, Chan Y, Fok T. Respiratory function in patients with thalassaemia major: relation with iron overload. Arch Dis Childhood. 2002;87(4):328-330.
- Kanj N, Shamseddine A, Gharzeddine W, et al. Relation of ferritin levels to pulmonary function in patients with thalassemia major and the acute effects of transfusion. Eur J Haematol. 2000;64(6):396-400.
- Chan KC, Au CT, Leung AW, et al. Pulmonary function in patients with transfusion-dependent thalassemia and its associations with iron overload. Scientific Rep. 2023;13(1):3674.
Details
| Primary Language | English |
|---|---|
| Subjects | Haematology |
| Journal Section | Research Articles |
| Authors | |
| Publication Date | March 8, 2024 |
| Submission Date | January 16, 2024 |
| Acceptance Date | February 11, 2024 |
| Published in Issue | Year 2024 Volume: 6 Issue: 2 |
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