Correlation Between Adherence to Iron Chelation Therapy and Serum Ferritin Levels in Patients with Thalassemia Major at Gotong Royong Hospital, Surabaya, Indonesia

Authors

  • Yustisia Amalia Blood Bank Technology Study Program, Faculty of Health Sciences, Universitas Dr. Soetomo, Surabaya, Indonesia
  • Miftahul Khairoh Midwifery Study Program, Faculty of Health Sciences, Universitas Dr. Soetomo, Surabaya, Indonesia
  • Arkha Rosyaria Badrus Midwifery Study Program, Faculty of Health Sciences, Universitas Dr. Soetomo, Surabaya, Indonesia

DOI:

https://doi.org/10.26911/theijmed.2026.11.3.968

Abstract

Background: Thalassemia major requires lifelong regular blood transfusions, which may lead to iron overload and increased serum ferritin levels. Iron chelation therapy is needed to control ferritin levels; However, its success is influenced by patient adherence. This study aimed to analyze the correlation between adherence to iron chelation therapy and serum ferritin levels in patients with thalassemia major.

Subjects and Method: A cross-sectional study involved 35 patients with thalassemia major who underwent regular blood transfusions and received iron chelation therapy. Adherence was assessed using an eight-item adherence questionnaire, while serum ferritin levels were obtained from labora­tory and medical records. Data were analyzed using descriptive statistics and Spearman’s rank correlation test.

Results: Most respondents had moderate adherence to iron chelation therapy (16 patients; 45.7%), followed by high adherence (11 patients; 31.4%) and low adherence (8 patients; 22.9%). Eighteen patients (51.4%) had very high serum ferritin levels, 13 patients (37.1%) had elevated levels, and 4 patients (11.4%) had controlled levels. Spearman’s rank correlation analysis demonstrated a significant moderate negative correlation between adherence to iron chelation therapy and serum ferritin levels (r=−0.51; p=0.002).

Conclusion: Adherence to iron chelation therapy was significantly and negatively correlated with serum ferritin levels in patients with thalassemia major. Higher adherence was associated with lower serum ferritin levels.

Keywords:

iron chelation therapy, serum ferritin, thalassemia major, blood transfusion

Published

2026-07-10

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Issue

Vol.11 No.3 (2026)

Section

Articles

References

Borgna-Pignatti C, Cappellini MD, De Stefano P, Del Vecchio GC, Forni GL, Gamberini MR, et al. (2005). Survival and complications in thalassemia. Ann N Y Acad Sci. 1054:40–47. https://doi.org/10.1196/annals.1345.006.

Borgna-Pignatti C, Rugolotto S, De Stefano P, Piga A, Di Gregorio F, Gamberini MR, et al. (1998). Survival and disease complications in thalassemia major. Ann N Y Acad Sci. 850:227–231. https://doi.org/10.1111/j.1749-6632.1998.tb10479.x.

Bou-Fakhredin R, Bazarbachi AH, Chaya B, Sleiman J, Cappellini MD, Taher AT (2017). Iron overload and chelation therapy in non-transfusion dependent thalassemia. Int J Mol Sci. 18(12):2778. https://doi.org/10.3390/ijms18122778.

Brittenham GM, Griffith PM, Nienhuis AW, McLaren CE, Young NS, Tucker EE, et al. (1994). Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major. N Engl J Med. 331(9):567–573. https://doi.org/10.1056/NEJM199409013310902.

Cao A, Galanello R (2010). Beta-thalassemia. Genet Med. 12(2):61–76. https://doi.org/10.1097/GIM.0b013e3181cd68ed.

Cappellini MD, Cohen A, Piga A, Bejaoui M, Perrotta S, Agaoglu L, et al. (2006). A phase 3 study of deferasirox (ICL670), a once-daily oral iron chelator, in patients with β-thalassemia. Blood. 107(9):3455–3462. https://doi.org/10.1182/blood-2005-08-3430.

Cappellini MD, Porter J, El-Beshlawy A, Li CK, Seymour JF, Elalfy M, et al. (2010). Tailoring iron chelation by iron intake and serum ferritin: the prospective EPIC study of deferasirox in 1744 patients with transfusion-dependent anemias. Haematologica. 95(4):557–566. https://doi.org/10.3324/haematol.2009.014696.

Delea TE, Edelsberg J, Sofrygin O, Thomas SK, Baladi JF, Phatak PD, et al. (2007). Consequences and costs of noncompliance with iron chelation therapy in patients with transfusion-dependent thalassemia: a literature review. Transfusion. 47(10):1919–1929. https://doi.org/10.1111/j.1537-2995.2007.01416.x.

Farmakis D, Porter J, Taher A, Cappellini MD, Angastiniotis M, Eleftheriou A (2022). 2021 Thalassaemia International Federation Guidelines for the Management of Transfusion-dependent Thalassemia. HemaSphere. 6(8): e732. https://doi.org/10.1097/HS9.0000000000000732.

Galanello R, Origa R (2010). Beta-thalassemia. Orphanet J Rare Dis. 5:11. https://doi.org/10.1186/1750-1172-5-11.

Galanello R, Piga A, Forni GL, Bertrand Y, Foschini ML, Bordone E, et al. (2006). Phase II clinical evaluation of deferasirox, a once-daily oral chelating agent, in pediatric patients with beta-thalassemia major. Haematologica. 91(10):1343–1351. https://pubmed.ncbi.nlm.nih.gov/17018383/.

Haghpanah S, Zarei T, Zahedi Z, Karimi M (2014). Compliance and satisfaction with deferasirox compared with deferoxamine in patients with transfusion-dependent beta-thalassemia. Hematology. 19(4):187–191. https://doi.org/10.1179/1607845413y.0000000121.

Maggio A, D'Amico G, Morabito A, Capra M, Ciaccio C, Cianciulli P, et al. (2002). Deferiprone versus deferoxamine in patients with thalassemia major: a randomized clinical trial. Blood Cells Mol Dis. 28(2):196–208. https://doi.org/10.1006/bcmd.2002.0510.

Maggio A, Vitrano A, Capra M, Cuccia L, Gagliardotto F, Filosa A, et al. (2009). Long-term sequential deferiprone-deferoxamine versus deferiprone alone for thalassemia major patients: a randomized clinical trial. Br J Haematol. 145(2):245–254. https://doi.org/10.1111/j.1365-2141.2009.07609.x.

Mobarra N, Shanaki M, Ehteram H, Nasiri H, Sahmani M, Saeidi M, et al. (2016). A review on iron chelators in treatment of iron overload syndromes. Int J Hematol Oncol Stem Cell Res. 10(4): 239–247. https://pmc.ncbi.nlm.nih.gov/articles/PMC5139945/.

Modell B, Darlison M (2008). Global epidemiology of hemoglobin disorders and derived service indicators. Bull World Health Organ. 86(6):480–487. https://doi.org/10.2471/BLT.06.036673.

Moukalled NM, Bou-Fakhredin R, Taher AT (2018). Deferasirox: over a decade of experience in thalassemia. Mediterr J Hematol Infect Dis. 10(1):e2018066. https://doi.org/10.4084/mjhid.2018.066.

Olivieri NF, Brittenham GM, Matsui D, Berkovitch M, Blendis LM, Cameron RG, et al. (1995). Iron-chelation therapy with oral deferiprone in patients with thalassemia major. N Engl J Med. 332(14):918–922. https://doi.org/10.1056/NEJM199504063321404.

Olivieri NF, Nathan DG, MacMillan JH, Wayne AS, Liu PP, McGee A, et al. (1994). Survival in medically treated patients with homozygous beta-thalassemia. N Engl J Med. 331(9): 574–578. https://doi.org/10.1056/NEJM199409013310903.

Pennell DJ, Berdoukas V, Karagiorga M, Ladis V, Piga A, Aessopos A, et al. (2006). Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. Blood. 107(9):3738–3744. https://doi.org/10.1182/blood-2005-07-2948.

Pennell DJ, Porter JB, Piga A, Lai YR, El-Beshlawy A, Elalfy M, et al. (2014). A 1-year randomized controlled trial of deferasirox versus deferoxamine for myocardial iron removal in beta-thalassemia major. Blood. 123(10):1447–1454. https://doi.org/10.1182/blood-2013-04-497842.

Pepe A, Meloni A, Rossi G, Cuccia L, D'Ascola DG, Santodirocco M, et al. (2013). Cardiac and hepatic iron and ejection fraction in thalassemia major: multicentre prospective comparison of combined deferiprone and deferoxamine therapy against deferiprone or deferoxamine monotherapy. J Cardiovasc Magn Reson. 15:1. https://doi.org/10.1186/1532-429X-15-1.

Piga A, Longo F, Origa R, Roggero S, Pinna F, Zappu A, et al. (2014). Deferasirox for cardiac siderosis in β-thalassemia major: a multicentre, open-label, prospective study. Br J Haematol. 167(3): 423–426. https://doi.org/10.1111/bjh.12987.

Pinto VM, Forni GL (2020). Management of iron overload in beta-thalassemia patients: clinical practice update based on case series. Int J Mol Sci. 21(22):8771. https://doi.org/10.3390/ijms21228771.

Poggiali E, Cassinerio E, Zanaboni L, Cappellini MD (2012). An update on iron chelation therapy. Blood Transfus. 10(4):411–422. https://doi.org/10.2450/2012.0008-12.

Porter JB (2001). Practical management of iron overload. Br J Haematol. 115(2): 239–252. https://doi.org/10.1046/j.1365-2141.2001.03195.x.

Porter JB, Garbowski MW (2014). The pathophysiology of transfusional iron overload. Hematol Oncol Clin North Am. 28(4):683–701. https://doi.org/10.1016/j.hoc.2014.04.003.

Reddy PS, Locke M, Badawy SM (2022). A systematic review of adherence to iron chelation therapy among children and adolescents with thalassemia. Ann Med. 54(1):326–342. https://doi.org/10.1080/07853890.2022.2028894.

Rofail D, Abetz L, Viala M, Gait C, Baladi JF, Payne KA (2009). Satisfaction and adherence in patients with iron overload receiving iron chelation therapy as assessed by a newly developed patient instrument. Value Health. 12(1):109–117. https://doi.org/10.1111/j.1524-4733.2008.00390.x.

Rund D, Rachmilewitz E (2005). Beta-thalassemia. N Engl J Med. 353(11):1135–1146. https://doi.org/10.1056/NEJMra050436.

Saliba AN, Harb AR, Taher AT (2015). Iron chelation therapy in transfusion-dependent thalassemia patients: current strategies and future directions. J Blood Med. 6:197–209. https://doi.org/10.2147/JBM.S72463.

Scalone L, Mantovani LG, Krol M, Rofail D, Ravera S, Bisconte MG, et al. (2008). Costs, quality of life, treatment satisfaction and compliance in patients with beta-thalassemia major undergoing iron chelation therapy: the ITHACA study. Curr Med Res Opin. 24(7):1905–1917. https://doi.org/10.1185/03007990802160834.

Sidhu S, Kakkar S, Dewan P, Bansal N, Sobti PC (2021). Adherence to iron chelation therapy and its determinants. Int J Hematol Oncol Stem Cell Res. 15(1): 27–34. https://doi.org/10.18502/ijhoscr.v15i1.5247.

Taher AT, Musallam KM, Cappellini MD (2021). β-Thalassemias. N Engl J Med. 384(8):727–743. https://doi.org/10.1056/NEJMra2021838.

Taher AT, Porter JB, Viprakasit V, Kattamis A, Chuncharunee S, Sutcharitchan P, et al. (2012). Deferasirox reduces iron overload significantly in nontransfusion-dependent thalassemia: one-year results from a prospective, randomized, double-blind, placebo-controlled study. Blood. 120(5):970–977. https://doi.org/10.1182/blood-2012-02-412692.

Taher AT, Saliba AN (2017). Iron overload in thalassemia: different organs at different rates. Hematology Am Soc Hematol Educ Program. 2017(1):265–271. https://doi.org/10.1182/asheducation-2017.1.265.

Tanner MA, Galanello R, Dessi C, Smith GC, Westwood MA, Agus A, et al. (2007). A randomized, placebo-controlled, double blind trial of the effect of combined therapy with deferoxamine and deferiprone on myocardial iron in thalassemia major using cardiovascular magnetic resonance. Circulation. 115(14):1876–1884. https://doi.org/10.1161/CIRCULATIONAHA.106.648790.

Telfer PT, Prescott E, Holden S, Walker M, Hoffbrand AV, Wonke B (2000). Hepatic iron concentration combined with long-term monitoring of serum ferritin to predict complications of iron overload in thalassaemia major. Br J Haematol. 110(4):971–977. https://doi.org/10.1046/j.1365-2141.2000.02298.x.

Trachtenberg F, Vichinsky E, Haines D, Pakbaz Z, Mednick L, Sobota A, et al. (2011). Iron chelation adherence to deferoxamine and deferasirox in thalassemia. Am J Hematol. 86(5):433–436. https://doi.org/10.1002/ajh.21993.

Weatherall DJ, Clegg JB (2001). Inherited haemoglobin disorders: an increasing global health problem. Bull World Health Organ. 79(8):704–712. https://pmc.ncbi.nlm.nih.gov/articles/PMC2566499/.