Comparison of Blood Transfusion Plus Chelation Therapy and Bone Marrow Transplantation in Patients with β-Thalassemia: Application of SF-36, EQ-5D, and Visual Analogue Scale Measures

Document Type : Original Article


1 Health Economics Research Unit, Institute of Applied Health Sciences, University of Aberdeen, Aberdeen, UK

2 Health Economics Department, School of Management and Information Sciences, Shiraz University of Medical Sciences, Shiraz, Iran

3 Department of Health Services Management, School of Health Management and Information Sciences, Iran University of Medical Sciences, Tehran, Iran

4 Health Management and Economics Research Center, Iran University of Medical Sciences, Tehran, Iran

5 Department of Internal Medicine, BMT Unit, Hematology Research Center, and Organ Transplant Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran

6 Department of Health Management and Economics, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran


β-Thalassemia is a prevalent genetic disease in Mediterranean countries. The most common treatments for this disease are blood transfusion plus iron chelation (BTIC) therapy and bone marrow transplantation (BMT). Patients using these procedures experience different health-related quality of life (HRQoL). The purpose of the present study was to measure HRQoL in these patients using 2 different multiattribute quality of life (QoL) scales.
In this cross-sectional study, data were gathered using 3 instruments: a socio-demographic questionnaire, EQ-5D, and SF-36. A total of 196 patients with β-thalassemia were randomly selected from 2 hospitals in Shiraz (Southern Iran). Data were analyzed using logistic regression and multiple regression models to identify factors that affect the patients’ HRQoL.
The average EQ-5D index and EQ visual analog scale (VAS) scores were 0.86 (95% CI: 0.83–0.89) and 71.85 (95% CI: 69.13–74.58), respectively. Patients with BMT reported significantly higher EQ VAS scores (83.27 vs 68.55, respectively). The results showed that patients who lived in rural area and patients with BMT reported higher EQ VAS scores (rural; β=10.25, P=.006 and BMT; β=11.88, P=.000). As well, SF-36 between 2 groups of patients were statistically significant in physical component scale (PCS).
Patients in the BMT group experienced higher HRQoL in both physical and mental aspects compared to those in the BTIC group. More studies are needed to assess the relative cost-effectiveness of these methods in developing countries


Main Subjects

  1. Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008;86:480-487.
  2. Karami H, Kosaryan M, Abolghasemi H, et al. Deferiprone plus deferoxamine versus deferoxamine iron chalation in beta thalassemia major. Sci J Iran Blood Transfus Organ. 2011;7(4):227-234.
  3. Imani E, Asadi Nooghabi F, Hosseini Teshnizi S, Yosefi P, Salari F. Comparison quality of life in patients with thalassemia major based on participating in group activities, Bandar Abbas. Sci J Iran Blood Transfus Organ. 2013;10(2):198-206.
  4. Kasper D, Fauci A, Longo D, Braunwald E, Hauser S, Jameson J. Harrison’s Principles of Internal Medicine: McGraw-Hill; 2005.
  5. Keshtkaran A, Javanbakht M, Salavati S, Mashayekhi A, Karimi M, Nuri B. Cost-utility analysis of oral deferasirox versus infusional deferoxamine in transfusion-dependent beta-thalassemia patients. Transfusion. 2013;53(8):1722-1729. doi:10.1111/trf.12024
  6. Ministry of Health. Comprehensive guidelines and educational materials national prevention program of beta thalassemia. Tehran: Center of publishing sound; 2004.
  7. Thavorncharoensap M, Torcharus K, Nuchprayoon I, Riewpaiboon A, Indaratna K, Ubol BO. Factors affecting health-related quality of life in Thai children with thalassemia. BMC Blood Disord. 2010;10:1. doi:10.1186/1471-2326-10-1
  8. Khani H‎, Majdi MR‎, Azad Marzabadi E‎, Montazeri A‎, Ghorbani A‎, Ramezani M‎. Quality of life in Iranian Beta-thalassemia major patients of ‎southern coastwise of the Caspian Sea. J Behav Sci. 2009;2(4):325-332.
  9. Cheuk DK, Mok AS, Lee AC, et al. Quality of life in patients with transfusion-dependent thalassemia after hematopoietic SCT. Bone Marrow Transplant. 2008;42(5):319-327. doi:10.1038/bmt.2008.165
  10. Shaligram D, Girimaji S, Chaturvedi S. Psychological problems and quality of life in children with thalassemia. Indian J Pediatr. 2007;74:727-730.
  11. Mantovani LG, Scalone L, Ravera S, Rofail D, Cappellini MD. Quality of Life and Satisfaction with Iron Chelation Therapy in Patients with Beta Thalassemia Major: Results from the ITHACA Study. ASH Annual Meeting Abstracts. 2006;108(11):3344.
  12. Allahyari A, Alhany F, Kazemnejad A, Izadyar M. The effect of family-centered empowerment model on the Quality of Life of school-age B-thalassemic children. Iranian Journal of Pediatrics. 2006;16(4):455-461.
  13. Uyl-de Groot CA, Buijt I, Gloudemans IJ, Ossenkoppele GJ, Berg HP, Huijgens PC. Health related quality of life in patients with multiple myeloma undergoing a double transplantation. Eur J Haematol. 2005;74(2):136-143. doi:10.1111/j.1600-0609.2004.00346.x
  14. Lee AJ, Morgan CL, Conway P, Currie CJ. Characterisation and comparison of health-related quality of life for patients with renal failure. Curr Med Res Opin. 2005;21(11):1777-1783. doi:10.1185/030079905x65277
  15. Javanbakht M, Abolhasani F, Mashayekhi A, Baradaran HR, Jahangiri Noudeh Y. Health related quality of life in patients with type 2 diabetes mellitus in Iran: a national survey. PLoS One. 2012;7(8):e44526. doi:10.1371/journal.pone.0044526
  16. Hadi N, Karami D, Montazeri  A. Health-related quality of life in major thalassemic patients. journal of the Iranian Institute for Health Sciences Research. 2009;8(4):387-393.
  17. Baraz Pordanjani S, Zare K, Pedram M, Pakbaz Z. Comparison of quality of life of thalassemic children with their parents. Sci Med J. 2010;8(4):456-462.
  18. Haghpanah S, Nasirabadi S, Ghaffarpasand F, et al. Quality of life among Iranian patients with beta-thalassemia major using the SF-36 questionnaire. Sao Paulo Med J. 2013;131(3):166-172.
  19. Safizadeh H, Farahmandinia Z, Nejad SS, Pourdamghan N, Araste M. Quality of life in patients with thalassemia major and intermedia in Kerman-Iran. Mediterr J Hematol Infect Dis. 2012;4(1):e2012058. doi:10.4084/mjhid.2012.058
  20. Azarkeivan A, Hajibeigi B, Alavian SM, Lankarani MM, Assari S. Associates of poor physical and mental health-related quality of life in beta thalassemia-major/intermedia. J Res Med Sci. 2009;14(6):349-355.
  21. Montazeri A, Goshtasebi A, Vahdaninia M, Gandek B. The Short Form Health Survey (SF-36): translation and validation study of the Iranian version. Qual Life Res. 2005;14(3):875-882.
  22. Goudarzi R, Akbari Sari A, Zeraati H, Rashidian A, Mohammad K. Population-based preference weights for EQ-5D Health States using visual analogue scale (VAS) in Iran. Iranian Red Crescent Medical Journal 2015; forthcoming.
  23. Adib-Hajbaghery M, Abasinia M. Assessing Quality of life of elders with femoral neck fractures, using SF36 and EQ5D. Iranian Journal of Nursing Research. 2010;4(15):71-79.
  24. Sobota A, Yamashita R, Xu Y, et al. Quality of life in thalassemia: a comparison of SF-36 results from the thalassemia longitudinal cohort to reported literature and the US norms. Am J Hematol. 2011;86(1):92-95. doi:10.1002/ajh.21896
  25. Ghavamzadeh A, Nasseri P, Eshraghian MR, et al. Prognostic factors in bone marrow transplantation for beta thalassemia major: experiences from Iran. Bone Marrow Transplant. 1998;22(12):1167-1169. doi:10.1038/sj.bmt.1701509
  26. Sruamsiri R, Chaiyakunapruk N, Pakakasama S, et al. Cost utility analysis of reduced intensity hematopoietic stem cell transplantation in adolescence and young adult with severe thalassemia compared to hypertransfusion and iron chelation program. BMC Health Serv Res. 2013;13:45. doi:10.1186/1472-6963-13-45
  27. Dahlui M, Hishamshah MI, Rahman AJ, Aljunid SM. Quality of life in transfusion-dependent thalassaemia patients on desferrioxamine treatment. Singapore Med J. 2009;50(8):794-799.
  28. Yengil E, Acipayam C, Kokacya MH, Kurhan F, Oktay G, Ozer C. Anxiety, depression and quality of life in patients with beta thalassemia major and their caregivers. Int J Clin Exp Med. 2014;7(8):2165-2172.
  29. Lawson SE, Roberts IA, Amrolia P, Dokal I, Szydlo R, Darbyshire PJ. Bone marrow transplantation for beta-thalassaemia major: the UK experience in two paediatric centres. Br J Haematol. 2003;120(2):289-295.