The Global survival rate of patients with beta thalassemia major: a systematic review and meta-analysis
DOI:
https://doi.org/10.22317/jcms.v7i2.903Keywords:
survival rate, beta thalassemia major, systematic review, meta-analysisAbstract
Objectives: Thalassemia is a public health challenge in the countries entitled belt thalassemia but there is no pooled estimated of the survival rate on thalassemia major patients. The aim of this meta-analysis was to evaluate the pooled 10-, 15-, 20-, and 30-year survival rates of the patients with beta thalassemia major around the world.
Methods: A comprehensive literature search of five international databases including Medline/PubMed, Scopus, Embase, Web of knowledge and ProQuest was conducted to identify studies reporting survival rate of beta thalassemia major until March 2020.
Results: From 714 retrieved studies, seven studies with 8777 subjects included in the meta-analysis. Base on random effect model, the 10-, 15-, 20-, and 30-year survival rates were 98.39, 95.07, 90.41, and 82.93 percent, respectively.
Conclusions: This meta-analysis provided acceptable results for estimating survival rate of beta-thalassemia compared to other studies. Hence, these results can be effectively used to develop and implement prevention and treatment interventions for policy makers.
References
1. Aessopos A, Farmakis D, Karagiorga M, Voskaridou E, Loutradi A, Hatziliami A, et al. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood. 2001;97(11):3411-6.
2. Olivieri NF. The beta-thalassemias. N Engl J Med. 1999;341(2):99-109.
3. Wang HC, Hsieh LL, Liu YC, Hsiao HH, Lin SK, Tsai WC, et al. The epidemiologic transition of thalassemia and associated hemoglobinopathies in southern Taiwan. Annals of hematology. 2017;96(2):183-8.
4. Borgna-Pignatti C, Cappellini MD, De Stefano P, Del Vecchio GC, Forni GL, Gamberini MR, et al. Survival and complications in thalassemia. Annals of the New York Academy of Sciences. 2005;1054:40-7.
5. Zafari M, Kosaryan M, Gill P, Alipour A, Shiran M, Jalalli H, et al. Non-invasive prenatal diagnosis of beta-thalassemia by detection of the cell-free fetal DNA in maternal circulation: a systematic review and meta-analysis. Annals of hematology. 2016;95(8):1341-50.
6. Borgna-Pignatti C, Rugolotto S, De Stefano P, Zhao H, Cappellini MD, Del Vecchio GC, et al. Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine. Haematologica. 2004;89(10):1187-93.
7. Ladis V, Chouliaras G, Berdousi H, Kanavakis E, Kattamis C. Longitudinal study of survival and causes of death in patients with thalassemia major in Greece. Annals of the New York Academy of Sciences. 2005;1054:445-50.
8. Tripathi P, Pati HP, Mahapatra M, Tyagi S, Ahuja A, Saxena R. Utility of Labile Plasma Iron Assay in Thalassemia Major Patients. Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion. 2019;35(2):272-7.
9. Zamani R, Khazaei S, Rezaeian S. Survival analysis and its associated factors of Beta thalassemia major in hamadan province. Iranian journal of medical sciences. 2015;40(3):233-9.
10. Wu H-P, Lin C-L, Chang Y-C, Wu K-H, Lei R-L, Peng C-T, et al. Survival and complication rates in patients with thalassemia major in Taiwan. Pediatric Blood & Cancer. 2017;64(1):135-8.
11. Ansari-Moghaddam A, Adineh HA, Zareban I, Mohammadi M, Maghsoodlu M. The survival rate of patients with beta-thalassemia major and intermedia and its trends in recent years in Iran. Epidemiol Health. 2018;40(0):e2018048-0.
12. Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance. 2008;10(1):42-.
13. Pakbaz Z, Fischer R, Treadwell M, Yamashita R, Fung EB, Calvelli L, et al. A simple model to assess and improve adherence to iron chelation therapy with deferoxamine in patients with thalassemia. Annals of the New York Academy of Sciences. 2005;1054:486-91.
14. Porter JB, Davis BA. Monitoring chelation therapy to achieve optimal outcome in the treatment of thalassaemia. Best practice & research Clinical haematology. 2002;15(2):329-68.
15. Moher D, Shamseer L, Clarke M, Ghersi D, Liberati A, Petticrew M, et al. Preferred reporting items for systematic review and meta-analysis protocols (PRISMA-P) 2015 statement. Systematic reviews. 2015;4(1):1-.
16. Penson DF, Krishnaswami S, Jules A, Seroogy JC, McPheeters ML. Evaluation and treatment of cryptorchidism. 2012.
17. Nikbakht HA, Hassanipour S, Shojaie L, Vali M, Ghaffari-Fam S, Ghelichi-Ghojogh M, et al. Survival Rate of Colorectal Cancer in Eastern Mediterranean Region Countries: A Systematic Review and Meta-Analysis. Cancer Control. 2020;27(1):1073274820964146.
18. Hassanipour S, Vali M, Gaffari-Fam S, Nikbakht HA, Abdzadeh E, Joukar F, et al. The survival rate of hepatocellular carcinoma in Asian countries: a systematic review and meta-analysis. EXCLI J. 2020;19:108-30.
19. Hassanipour S, Delam H, Arab-Zozani M, Abdzadeh E, Hosseini SA, Nikbakht HA, et al. Survival Rate of Prostate Cancer in Asian Countries: A Systematic Review and Meta-Analysis. Ann Glob Health. 2020;86(1):2.
20. Kosaryan M, Vahidshahi K, Karami H, Forootan MA, Ahangari M. Survival of thalassemic patients referred to the Boo Ali Sina Teaching Hospital, Sari, Iran. Hemoglobin. 2007;31(4):453-62.
21. Roudbari M, Soltani-Rad M, Roudbari S. The survival analysis of beta thalassemia major patients in South East of Iran. Saudi Medical Journal. 2008;29(7):1031-5.
22. Rajaeefard A, Hajipour M, Tabatabaee HR, Hassanzadeh J, Rezaeian S, Moradi Z, et al. Analysis of survival data in thalassemia patients in Shiraz, Iran. Epidemiol Health. 2015;37:e2015031.
23. Ansari-Moghaddam A, Adineh HA, Zareban I, Mohammadi M, Maghsoodlu M. The survival rate of patients with beta-thalassemia major and intermedia and its trends in recent years in Iran. Epidemiol Health. 2018;40:e2018048.
24. Di Bartolomeo P, Santarone S, Di Bartolomeo E, Olioso P, Bavaro P, Papalinetti G, et al. Long-term results of survival in patients with thalassemia major treated with bone marrow transplantation. American Journal of Hematology. 2008;83(7):528-30.
25. Telfer P, Coen PG, Christou S, Hadjigavriel M, Kolnakou A, Pangalou E, et al. Survival of medically treated thalassemia patients in Cyprus. Trends and risk factors over the period 1980-2004. Haematologica. 2006;91(9):1187-92.
26. Schneck A. Examining publication bias-a simulation-based evaluation of statistical tests on publication bias. PeerJ. 2017;5:e4115.
27. Telfer P, Coen PG, Christou S, Hadjigavriel M, Kolnakou A, Pangalou E, et al. Survival of medically treated thalassemia patients in Cyprus. Trends and risk factors over the period 1980-2004. Haematologica. 2006;91(9):1187-92.
28. Hassanzadeh J, Mirahmadizadeh A, Karimi M, Veisani Y, Rezaeian S. Trends in 5-, 10-, 20-, and 30-year survival rates of beta-thalassemia patients in Southern Iran, 1995-2016: A retrospective cohort study. Journal of public health research. 2017;6(3).
29. Charafeddine K, Isma’eel H, Charafeddine M, Inati A, Koussa S, Naja M, et al. Survival and complications of beta-thalassemia in Lebanon. Acta haematologica. 2008;120(2):112-6.
30. Caocci G, Orofino MG, Vacca A, Piroddi A, Piras E, Addari MC, et al. Long-term survival of beta thalassemia major patients treated with hematopoietic stem cell transplantation compared with survival with conventional treatment. American journal of hematology. 2017;92(12):1303-10.
31. Miri M, Tabrizi Namini M, Hadipour Dehshal M, Sadeghian Varnosfaderani F, Ahmadvand A, Yousefi Darestani S, et al. Thalassemia in Iran in last twenty years: the carrier rates and the births trend. Iranian journal of blood and cancer. 2013;6(1):11-7.
32. Hassanzadeh J, Mirahmadizadeh A, Karimi M, Rezaeian S. Delay in diagnosis of hemoglobulinopathies (thalassemia, sickle cell anemia): a need for management of thalassemia programs. Iranian Journal of Pediatrics. 2017;27(2).
33. Hadipour Dehshal M, Ahmadvand A, Yousefi Darestani S, Manshadi M, Abolghasemi H. Secular trends in the national and provincial births of new thalassemia cases in Iran from 2001 to 2006. Hemoglobin. 2013;37(2):124-37.
34. Efthimiadis GK, Hassapopoulou HP, Tsikaderis DD, Karvounis HI, Giannakoulas G, Parharidis GE. Cardiac determinants of survival in beta-thalassemia. Haematologica. 2006;91(12 Suppl):Elt11.
35. Di Bartolomeo P, Santarone S, Di Bartolomeo E, Olioso P, Bavaro P, Papalinetti G, et al. Long-term results of survival in patients with thalassemia major treated with bone marrow transplantation. American journal of hematology. 2008;83(7):528-30.
36. Wu HP, Lin CL, Chang YC, Wu KH, Lei RL, Peng CT, et al. Survival and complication rates in patients with thalassemia major in Taiwan. Pediatric blood & cancer. 2017;64(1):135-8.
37. Peng C-T, Tsai C-H, Wu K-H. Effects of chelation therapy on cardiac function improvement in thalassemia patients: literature review and the Taiwanese experience. Hemoglobin. 2008;32(1-2):49-62.
38. Peng C-T, Chang J-S, Wang L-Y, Chiou S-S, Hsiao C-C, Wang S-C, et al. Update on thalassemia treatment in Taiwan, including bone marrow transplantation, chelation therapy, and cardiomyopathy treatment effects. Hemoglobin. 2009;33(5):304-11.
39. Huang Y-C, Chang J-S, Wu K-H, Peng C-T. Regression of myocardial dysfunction after switching from desferrioxamine to deferiprone therapy in β-thalassemia major patients. Hemoglobin. 2006;30(2):229-38.
2. Olivieri NF. The beta-thalassemias. N Engl J Med. 1999;341(2):99-109.
3. Wang HC, Hsieh LL, Liu YC, Hsiao HH, Lin SK, Tsai WC, et al. The epidemiologic transition of thalassemia and associated hemoglobinopathies in southern Taiwan. Annals of hematology. 2017;96(2):183-8.
4. Borgna-Pignatti C, Cappellini MD, De Stefano P, Del Vecchio GC, Forni GL, Gamberini MR, et al. Survival and complications in thalassemia. Annals of the New York Academy of Sciences. 2005;1054:40-7.
5. Zafari M, Kosaryan M, Gill P, Alipour A, Shiran M, Jalalli H, et al. Non-invasive prenatal diagnosis of beta-thalassemia by detection of the cell-free fetal DNA in maternal circulation: a systematic review and meta-analysis. Annals of hematology. 2016;95(8):1341-50.
6. Borgna-Pignatti C, Rugolotto S, De Stefano P, Zhao H, Cappellini MD, Del Vecchio GC, et al. Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine. Haematologica. 2004;89(10):1187-93.
7. Ladis V, Chouliaras G, Berdousi H, Kanavakis E, Kattamis C. Longitudinal study of survival and causes of death in patients with thalassemia major in Greece. Annals of the New York Academy of Sciences. 2005;1054:445-50.
8. Tripathi P, Pati HP, Mahapatra M, Tyagi S, Ahuja A, Saxena R. Utility of Labile Plasma Iron Assay in Thalassemia Major Patients. Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion. 2019;35(2):272-7.
9. Zamani R, Khazaei S, Rezaeian S. Survival analysis and its associated factors of Beta thalassemia major in hamadan province. Iranian journal of medical sciences. 2015;40(3):233-9.
10. Wu H-P, Lin C-L, Chang Y-C, Wu K-H, Lei R-L, Peng C-T, et al. Survival and complication rates in patients with thalassemia major in Taiwan. Pediatric Blood & Cancer. 2017;64(1):135-8.
11. Ansari-Moghaddam A, Adineh HA, Zareban I, Mohammadi M, Maghsoodlu M. The survival rate of patients with beta-thalassemia major and intermedia and its trends in recent years in Iran. Epidemiol Health. 2018;40(0):e2018048-0.
12. Modell B, Khan M, Darlison M, Westwood MA, Ingram D, Pennell DJ. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance. Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance. 2008;10(1):42-.
13. Pakbaz Z, Fischer R, Treadwell M, Yamashita R, Fung EB, Calvelli L, et al. A simple model to assess and improve adherence to iron chelation therapy with deferoxamine in patients with thalassemia. Annals of the New York Academy of Sciences. 2005;1054:486-91.
14. Porter JB, Davis BA. Monitoring chelation therapy to achieve optimal outcome in the treatment of thalassaemia. Best practice & research Clinical haematology. 2002;15(2):329-68.
15. Moher D, Shamseer L, Clarke M, Ghersi D, Liberati A, Petticrew M, et al. Preferred reporting items for systematic review and meta-analysis protocols (PRISMA-P) 2015 statement. Systematic reviews. 2015;4(1):1-.
16. Penson DF, Krishnaswami S, Jules A, Seroogy JC, McPheeters ML. Evaluation and treatment of cryptorchidism. 2012.
17. Nikbakht HA, Hassanipour S, Shojaie L, Vali M, Ghaffari-Fam S, Ghelichi-Ghojogh M, et al. Survival Rate of Colorectal Cancer in Eastern Mediterranean Region Countries: A Systematic Review and Meta-Analysis. Cancer Control. 2020;27(1):1073274820964146.
18. Hassanipour S, Vali M, Gaffari-Fam S, Nikbakht HA, Abdzadeh E, Joukar F, et al. The survival rate of hepatocellular carcinoma in Asian countries: a systematic review and meta-analysis. EXCLI J. 2020;19:108-30.
19. Hassanipour S, Delam H, Arab-Zozani M, Abdzadeh E, Hosseini SA, Nikbakht HA, et al. Survival Rate of Prostate Cancer in Asian Countries: A Systematic Review and Meta-Analysis. Ann Glob Health. 2020;86(1):2.
20. Kosaryan M, Vahidshahi K, Karami H, Forootan MA, Ahangari M. Survival of thalassemic patients referred to the Boo Ali Sina Teaching Hospital, Sari, Iran. Hemoglobin. 2007;31(4):453-62.
21. Roudbari M, Soltani-Rad M, Roudbari S. The survival analysis of beta thalassemia major patients in South East of Iran. Saudi Medical Journal. 2008;29(7):1031-5.
22. Rajaeefard A, Hajipour M, Tabatabaee HR, Hassanzadeh J, Rezaeian S, Moradi Z, et al. Analysis of survival data in thalassemia patients in Shiraz, Iran. Epidemiol Health. 2015;37:e2015031.
23. Ansari-Moghaddam A, Adineh HA, Zareban I, Mohammadi M, Maghsoodlu M. The survival rate of patients with beta-thalassemia major and intermedia and its trends in recent years in Iran. Epidemiol Health. 2018;40:e2018048.
24. Di Bartolomeo P, Santarone S, Di Bartolomeo E, Olioso P, Bavaro P, Papalinetti G, et al. Long-term results of survival in patients with thalassemia major treated with bone marrow transplantation. American Journal of Hematology. 2008;83(7):528-30.
25. Telfer P, Coen PG, Christou S, Hadjigavriel M, Kolnakou A, Pangalou E, et al. Survival of medically treated thalassemia patients in Cyprus. Trends and risk factors over the period 1980-2004. Haematologica. 2006;91(9):1187-92.
26. Schneck A. Examining publication bias-a simulation-based evaluation of statistical tests on publication bias. PeerJ. 2017;5:e4115.
27. Telfer P, Coen PG, Christou S, Hadjigavriel M, Kolnakou A, Pangalou E, et al. Survival of medically treated thalassemia patients in Cyprus. Trends and risk factors over the period 1980-2004. Haematologica. 2006;91(9):1187-92.
28. Hassanzadeh J, Mirahmadizadeh A, Karimi M, Veisani Y, Rezaeian S. Trends in 5-, 10-, 20-, and 30-year survival rates of beta-thalassemia patients in Southern Iran, 1995-2016: A retrospective cohort study. Journal of public health research. 2017;6(3).
29. Charafeddine K, Isma’eel H, Charafeddine M, Inati A, Koussa S, Naja M, et al. Survival and complications of beta-thalassemia in Lebanon. Acta haematologica. 2008;120(2):112-6.
30. Caocci G, Orofino MG, Vacca A, Piroddi A, Piras E, Addari MC, et al. Long-term survival of beta thalassemia major patients treated with hematopoietic stem cell transplantation compared with survival with conventional treatment. American journal of hematology. 2017;92(12):1303-10.
31. Miri M, Tabrizi Namini M, Hadipour Dehshal M, Sadeghian Varnosfaderani F, Ahmadvand A, Yousefi Darestani S, et al. Thalassemia in Iran in last twenty years: the carrier rates and the births trend. Iranian journal of blood and cancer. 2013;6(1):11-7.
32. Hassanzadeh J, Mirahmadizadeh A, Karimi M, Rezaeian S. Delay in diagnosis of hemoglobulinopathies (thalassemia, sickle cell anemia): a need for management of thalassemia programs. Iranian Journal of Pediatrics. 2017;27(2).
33. Hadipour Dehshal M, Ahmadvand A, Yousefi Darestani S, Manshadi M, Abolghasemi H. Secular trends in the national and provincial births of new thalassemia cases in Iran from 2001 to 2006. Hemoglobin. 2013;37(2):124-37.
34. Efthimiadis GK, Hassapopoulou HP, Tsikaderis DD, Karvounis HI, Giannakoulas G, Parharidis GE. Cardiac determinants of survival in beta-thalassemia. Haematologica. 2006;91(12 Suppl):Elt11.
35. Di Bartolomeo P, Santarone S, Di Bartolomeo E, Olioso P, Bavaro P, Papalinetti G, et al. Long-term results of survival in patients with thalassemia major treated with bone marrow transplantation. American journal of hematology. 2008;83(7):528-30.
36. Wu HP, Lin CL, Chang YC, Wu KH, Lei RL, Peng CT, et al. Survival and complication rates in patients with thalassemia major in Taiwan. Pediatric blood & cancer. 2017;64(1):135-8.
37. Peng C-T, Tsai C-H, Wu K-H. Effects of chelation therapy on cardiac function improvement in thalassemia patients: literature review and the Taiwanese experience. Hemoglobin. 2008;32(1-2):49-62.
38. Peng C-T, Chang J-S, Wang L-Y, Chiou S-S, Hsiao C-C, Wang S-C, et al. Update on thalassemia treatment in Taiwan, including bone marrow transplantation, chelation therapy, and cardiomyopathy treatment effects. Hemoglobin. 2009;33(5):304-11.
39. Huang Y-C, Chang J-S, Wu K-H, Peng C-T. Regression of myocardial dysfunction after switching from desferrioxamine to deferiprone therapy in β-thalassemia major patients. Hemoglobin. 2006;30(2):229-38.
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Published
2021-04-26
How to Cite
Soltani, M., Hassanipour, S., Veisani, Y., Darbandi, M., & Rezaiean, S. (2021). The Global survival rate of patients with beta thalassemia major: a systematic review and meta-analysis. Journal of Contemporary Medical Sciences, 7(2), 80–85. https://doi.org/10.22317/jcms.v7i2.903
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Review
