ACMJ
Anatolian Current Medical Journal

Anatolian Current Medical Journal (ACMJ) is an unbiased, peer-reviewed, and open access international medical journal. The Journal publishes interesting clinical and experimental research conducted in all fields of medicine, interesting case reports, and clinical images, invited reviews, editorials, letters, comments, and related knowledge.

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Original Article
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 : Thalassemia, respiration, iron load

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1. Sohn EY, Kato R, Noetzli LJ, et al. Exercise performance inthalassemia major: correlation with cardiac iron burden. Am JHematol. 2013;88(3):193-197.
2. Origa, R. β-Thalassemia. Genet Med. 2017;19(6):609-619.
3. Arora M, Chandra J, Suri JC, Narayan S, Dutta AK.Pulmonary function tests in beta thalassemia. Indian J Pediatr.2001;68(3):239-242.
4. Kanj N, Shamseddine A, Gharzeddine W, et al. Relation offerritin levels to pulmonary function in patients with thalassemiamajor and the acute effects of transfusion. Eur J Haematol.2000;64(6):396-400.
5. Kazazian Jr HH. The thalassemia syndromes: molecular basis andprenatal diagnosis in 1990. Semin Hematol. 1990;27(3):209-228.
6. Witzleben CL, Wyatt J. The effect of long survival on the pathologyof thalassaemia major. J Pathol Bacteriol. 1961;82(1):1-12.
7. Factor JM, Pottipatti SR, Rappoport I, Rosner IK, Lesser ML,Giardina PJ. Pulmonary function abnormalities in thalassemiamajor and the role of iron overload. Am J Respir Crit Care Med.1994;149(6):1570-1574.
8. Bacalo A, Kivity S, Heno N, Greif Z, Greif J, Topilsky M. Bloodtransfusion and lung function in children with thalassemiamajor. Chest. 1992;101(2):362-365.
9. Piatti G, Allegra L, Ambrosetti U, Cappellini MD, Turati F, FiorelliG. Beta-thalassemia and pulmonary function. Haematologica.1999;84(9):804-808.
10. Alyasin S, Moghtaderi M, Amin R, Kashef S, Karimi M. Pulmonaryfunction test in transfusion-dependent β-thalassemia majorpatients: a pilot study. Pediatr Hematol Oncol. 2011;28(4);329-333.
11. Cappell DF, Hutchison HE, Jowett M. Transfusional siderosis: theeffects of excessive iron deposits on the tissues. J Pathol Bacteriol.1975;74;245-264.
12. Papanikolaou G, Pantopoulos K. Iron metabolism and toxicity.Toxicol App Pharmacol. 2005;202(2):199-211.
13. Ooi GC, Khong PL, Lam WK, Trendell-Smith NJ, Tsang KWT.Pulmonary iron overload in thalassemia major presenting assmall airway disease. Acta Haematol. 2002;108(1):43-46.
14. Bourli E, Dimitriadou M, Economou M, et al. Restrictivepulmonary dysfunction and its predictors in young patients withβ-thalassaemia major. Pediatr Pulmonol. 2012;47(8):801-807.
15. Eldor A, Rachmilewitz EA. The hypercoagulable state inthalassemia. Blood. 2002;99(1):36-43.
16. Sumiyoshi A, Thakerngpol K, Sonakul D. Pulmonarymicrothromboemboli in thalassemic cases. Southeast Asian JTrop Med Public Health. 1992;23(Suppl 2):29-31.
17. Chatterjee R, Bajoria R. Critical appraisal of growth retardationand pubertal disturbances in thalassemia. Ann New York AcadSci. 2010;1202(1);100-114.
18. Li AM, Chan D, Li CK, Wong E, Chan Y, Fok T. Respiratoryfunction in patients with thalassaemia major: relation with ironoverload. Arch Dis Childhood. 2002;87(4):328-330.
19. Kanj N, Shamseddine A, Gharzeddine W, et al. Relation offerritin levels to pulmonary function in patients with thalassemiamajor and the acute effects of transfusion. Eur J Haematol.2000;64(6):396-400.
20. Chan KC, Au CT, Leung AW, et al. Pulmonary function in patientswith transfusion-dependent thalassemia and its associations withiron overload. Scientific Rep. 2023;13(1):3674.
Article available in :
Volume 6, Issue 2, 2024
Page : 139-143
https://doi.org/10.38053/acmj.1420858
Article Information
Submitted : 16 Oca 2024
Accepted : 11 Şub 2024
Corresponding Author :

Cem Selim: Department of Adult Hematology, Şanlıurfa Mehmet Akif İnan Training and Research Hospital, Şanlıurfa, Turkiye

[email protected]
Citation : Selim C, Çiftçiler R. The relationship between increased iron load and respiratory function tests in patients diagnosed with transfusion dependent thalassemia. Anatolian Curr Med J. 2024;6(2):139-143.

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