ASSESSMENT OF SERUM FERRITIN IN BETA THALASSEMIA MAJOR PATIENTS: INSIGHTS FROM A THALASSEMIA CENTRE IN PAKISTAN

Main Article Content

Wahid Ullah
Parveen Anjum
Attiya Sultana
Marium Khan
Rafia Amin

Keywords

Beta thalassemia major, Iron overload. Ferritin level, red blood cells (RBCs)

Abstract

Beta thalassemia major is the most common monogenic hereditary hemoglobinopathy which poses a major health burden in Pakistan. Regular transfusion of RBC’s (erythrocytes) required for survival of these patients which consequently leads to inevitable iron overload, which is demonstrated by elevated serum ferritin levels. Developing of iron excess may lead to organ damage and other life-threatening diseases. The aim of this study was to evaluate iron overload in beta thalassemia major patients in one of the thalassemia centers of Pakistan by means of their serum ferritin levels. The study was also conducted to approximate the current situation of iron overload in them. 155 blood samples of clinically diagnosed beta thalassemia major patients were collected from Jamila sultana foundation (JSF) for their serum ferritin estimation. Serum ferritin measurement was performed using indirect enzyme linked immune sorbent (ELISA) based serum ferritin assay kit. Demographics data of the patients were statistically analyzed by using SPSS 2.O and association between age, gender and serum ferritin levels were established. 85.4% of the beta thalassemia major patients showed very high ferritin levels. The mean serum ferritin level was found to be 4442 ± 2882 ng/ml. 44.4% patients had serum ferritin between 1000 to 2500 ng/ml, while 43.05% patients had values above 2500 ng/ml. Majority of the patients with high serum ferritin concentration (>2500 ng/mL) were aged more than 11 years. These levels reflect inadequate chelation and vulnerability to develop iron overload related complications. There is an urgent need to rationalize the chelation therapy and to create focus about the consequences of iron overload in these patients. The study showed elevated levels of serum ferritin beta thalassemia major patients which provide a normal bleak view.

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References

1. Riaz H, Riaz T, Khan MU, Aziz S, Ullah F, Rehman A, et al. Serum ferritin levels, socio-demographic factors and desferrioxamine therapy in multi-transfused thalassemia major patients at a government tertiary care hospital of Karachi, Pakistan. BMC research notes. 2011;4(1):1-5.
2. Knovich MA, Storey JA, Coffman LG, Torti SV, Torti FM. Ferritin for the clinician. Blood reviews. 2009;23(3):95-104.
3. Mishra AK, Tiwari A. Iron overload in beta thalassaemia major and intermedia patients. Maedica. 2013;8(4):328.
4. Shah N, Mishra A, Chauhan D, Vora C, Shah N. Study on effectiveness of transfusion program in thalassemia major patients receiving multiple blood transfusions at a transfusion centre in Western India. Asian journal of transfusion science. 2010;4(2):94.
5. Karunaratna A, Ranasingha JS, Mudiyanse RM. Iron overload in beta thalassemia major patients. Int J Blood Transfus Immunohematol. 2017;7:33-40.
6. Hows J, Hussein S, Hoffbrand A, Wickramasinghe S. Red cell indices and serum ferritin levels in children. Journal of Clinical Pathology. 1977;30(2):181.
7. Berdoukas V, Farmaki K, Carson S, Wood J, Coates T. Treating thalassemia major-related iron overload: the role of deferiprone. Journal of blood medicine. 2012:119-29.
8. Choudhry V, Pati H, Saxena A, Malaviya A. Deferiprone, efficacy and safety. The Indian Journal of Pediatrics. 2004;71:213-6.
9. Shamsian BS, Esfahani SA, Milani H, Akhlaghpoor S, Mojtahedzadeh S, Karimi A, et al. Magnetic resonance imaging in the evaluation of iron overload: a comparison of MRI, echocardiography and serum ferritin level in patients with β-thalassemia major. Clinical imaging. 2012;36(5):483-8.
10. Al Jaouni SK. Survival and disease complication of thalassemia major: experience of 14 years at King Abdulaziz University Hospital, Jeddah, KSA. Journal of King Abdulaziz University: Medical Sciences. 2010;98(277):1-20.
11. Nf O. Iron-chelating therapy and the treatment of thalassemia. Blood. 1997;89:739-61.
12. Ehsan H, Wahab A, Anwer F, Iftikhar R, Yousaf MN. Prevalence of transfusion transmissible infections in beta-thalassemia major patients in Pakistan: a systematic review. Cureus. 2020;12(8).
13. Prabhu R, Prabhu V, Prabhu R. Iron overload in beta thalassemia: a review. J Biosci Tech. 2009;1(1):20-31.
14. Brili SV, Castelanos SS, Aggeli CJ, Tentolouris CA, Pitsavos CE, Toutouzas PK, et al. The effect of iron overload in the hearts of patients with beta‐thalassemia. Clinical cardiology. 1997;20(6):541-6.
15. Karunaratna A, Ranasingha JS, Mudiyanse RM. Iron overload in beta thalassemia major patients. Int J Blood Transfus Immunohematol. 2017;7:33-40.
16. Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood, The Journal of the American Society of Hematology. 2011;118(13):3479-88.
17. Cao A, Galanello R. Beta-thalassemia. Genetics in medicine. 2010;12(2):61-76.
18. Cappellini MD. Exjade®(deferasirox, ICL670) in the treatment of chronic iron overload associated with blood transfusion. Therapeutics and clinical risk management. 2007;3(2):291-9.
19. Gutteridge JM, Rowley DA, Halliwell B. Superoxide-dependent formation of hydroxyl radicals in the presence of iron salts. Detection of ‘free’iron in biological systems by using bleomycin-dependent degradation of DNA. Biochemical Journal. 1981;199(1):263-5.
20. Bacon BR, Britton RS. The pathology of hepatic iron overload: a free radical–mediated process? Hepatology. 1990;11(1):127-37.
21. Hershko C, Konijn AM, Link G. Iron chelators for thalassaemia. British Journal of Haematology. 1998;101(3):399-406.
22. Vichinsky EP. Changing patterns of thalassemia worldwide. Annals of the New York Academy of Sciences. 2005;1054(1):18-24.
23. Weatherall D, Clegg JB. Inherited haemoglobin disorders: an increasing global health problem. Bulletin of the World Health Organization. 2001;79(8):704-12.
24. Asif N, Hassan K. Management of thalassemia in Pakistan. Journal of Islamabad Medical & Dental College. 2016;5(4):152-3.
25. Hashemi A, Ghilian R, Golestan M, Akhavan GM, Zare Z, Dehghani MA. The study of growth in thalassemic patients and its correlation with serum ferritin level. 2011.
26. Saka N, ŞüKür M, Bundak R, Anak S, Neyzi O, Gedikoglu G. Growth and puberty in thalassemia major. Journal of Pediatric Endocrinology and Metabolism. 1995;8(3):181-6.
27. Riaz H, Riaz T, Khan MU, Aziz S, Ullah F, Rehman A, et al. Serum ferritin levels, socio-demographic factors and desferrioxamine therapy in multi-transfused thalassemia major patients at a government tertiary care hospital of Karachi, Pakistan. BMC research notes. 2011;4(1):1-5.
28. Knovich MA, Storey JA, Coffman LG, Torti SV, Torti FM. Ferritin for the clinician. Blood reviews. 2009;23(3):95-104.
29. Mishra AK, Tiwari A. Iron overload in beta thalassaemia major and intermedia patients. Maedica. 2013;8(4):328.
30. Shah N, Mishra A, Chauhan D, Vora C, Shah N. Study on effectiveness of transfusion program in thalassemia major patients receiving multiple blood transfusions at a transfusion centre in Western India. Asian journal of transfusion science. 2010;4(2):94.
31. Hows J, Hussein S, Hoffbrand A, Wickramasinghe S. Red cell indices and serum ferritin levels in children. Journal of Clinical Pathology. 1977;30(2):181.
32. Berdoukas V, Farmaki K, Carson S, Wood J, Coates T. Treating thalassemia major-related iron overload: the role of deferiprone. Journal of blood medicine. 2012:119-29.
33. Choudhry V, Pati H, Saxena A, Malaviya A. Deferiprone, efficacy and safety. The Indian Journal of Pediatrics. 2004;71:213-6.
34. Shamsian BS, Esfahani SA, Milani H, Akhlaghpoor S, Mojtahedzadeh S, Karimi A, et al. Magnetic resonance imaging in the evaluation of iron overload: a comparison of MRI, echocardiography and serum ferritin level in patients with β-thalassemia major. Clinical imaging. 2012;36(5):483-8.
35. Al Jaouni SK. Survival and disease complication of thalassemia major: experience of 14 years at King Abdulaziz University Hospital, Jeddah, KSA. Journal of King Abdulaziz University: Medical Sciences. 2010;98(277):1-20.
36. Nf O. Iron-chelating therapy and the treatment of thalassemia. Blood. 1997;89:739-61.
37. Ehsan H, Wahab A, Anwer F, Iftikhar R, Yousaf MN. Prevalence of transfusion transmissible infections in beta-thalassemia major patients in Pakistan: a systematic review. Cureus. 2020;12(8).