Iron Overload Status in Patients with Non-transfusion-dependent Thalassemia in China

Overview

Journal Ther Adv Hematol

Specialty Hematology

Date 2022 Mar 24

PMID 35321211

Authors

Yumei Huang

Gaohui Yang

Man Wang

Xiaoyun Wei

Lingyuan Pan

Jiaodi Liu

Yu Lei

Peng

Liling Long

Yongrong Lai

Rongrong Liu

Affiliations

Soon will be listed here.

Abstract

Background: Iron overload is one of the main factors that increase morbidity and mortality in patients with non-transfusion dependent thalassemia (NTDT).

Aim: This study aimed at investigating the prevalence and severity of iron overload in Chinese NTDT patients.

Methods: we analyzed serum ferritin (SF), liver iron concentration (LIC) and cardiac T2* in 178 Chinese NTDT in this cross-sectional study.

Results: The median SF level was 996.00(27.15-19704.00) ng/ml and the median LIC value was 8.90(0.60-43.00) mg Fe/g dry weight (dw). The youngest patient with liver iron overload was 5 years old with 5.6 mg Fe/g dw in LIC. The median cardiac T2* was 33.06(7.46-75.08) ms. 6 patients had cardiac T2*⩽20ms. The patients with β thalassemia intermedia and HbE/β thalassemia showed a statistically significant lower Hb and higher values of SF and LIC than those of hemoglobin H disease patients. On multivariate logistic regression analysis, patients in ⩾ age 30-year old had a significant higher risk for iron overload (OR: 77.75, 95% CI: 8.76-690.49) in the age group. The detailed analysis of proportions of different LIC indicate in > 30-year old group, 76.8% patients suffered from moderate and severe LIC.

Conclusion: Our study provides a strong support for the novel findings that Chinese NTDT patients have a high prevalence of iron overload. The first assessment of MRI LIC should be performed as early as 5 years old. Then, NTDT patients > 30 years old may suffer with a high burden of iron overload.

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References

Weatherall D . The definition and epidemiology of non-transfusion-dependent thalassemia. Blood Rev. 2012; 26 Suppl 1:S3-6. DOI: 10.1016/S0268-960X(12)70003-6. View

Taher A, Musallam K, Cappellini M, Weatherall D . Optimal management of β thalassaemia intermedia. Br J Haematol. 2011; 152(5):512-23. DOI: 10.1111/j.1365-2141.2010.08486.x. View

Cheong J, Kim H, Lee K, Yoon S, Lee J, Park H . Deferasirox improves hematologic and hepatic function with effective reduction of serum ferritin and liver iron concentration in transfusional iron overload patients with myelodysplastic syndrome or aplastic anemia. Transfusion. 2013; 54(6):1542-51. DOI: 10.1111/trf.12507. View

Musallam K, Cappellini M, Wood J, Taher A . Iron overload in non-transfusion-dependent thalassemia: a clinical perspective. Blood Rev. 2012; 26 Suppl 1:S16-9. DOI: 10.1016/S0268-960X(12)70006-1. View

Saliba A, Taher A . Morbidities in non-transfusion-dependent thalassemia. Ann N Y Acad Sci. 2016; 1368(1):82-94. DOI: 10.1111/nyas.13083. View

Tantiworawit A, Charoenkwan P, Hantrakool S, Choeyprasert W, Sivasomboon C, Sanguansermsri T . Iron overload in non-transfusion-dependent thalassemia: association with genotype and clinical risk factors. Int J Hematol. 2016; 103(6):643-8. DOI: 10.1007/s12185-016-1991-5. View

Laosombat V, Viprakasit V, Chotsampancharoen T, Wongchanchailert M, Khodchawan S, Chinchang W . Clinical features and molecular analysis in Thai patients with HbH disease. Ann Hematol. 2009; 88(12):1185-92. DOI: 10.1007/s00277-009-0743-5. View

Taher A, Musallam K, Wood J, Cappellini M . Magnetic resonance evaluation of hepatic and myocardial iron deposition in transfusion-independent thalassemia intermedia compared to regularly transfused thalassemia major patients. Am J Hematol. 2010; 85(4):288-90. DOI: 10.1002/ajh.21626. View

Musallam K, Cappellini M, Wood J, Motta I, Graziadei G, Tamim H . Elevated liver iron concentration is a marker of increased morbidity in patients with β thalassemia intermedia. Haematologica. 2011; 96(11):1605-12. PMC: 3208677. DOI: 10.3324/haematol.2011.047852. View

10.

Lal A, Goldrich M, Haines D, Azimi M, Singer S, Vichinsky E . Heterogeneity of hemoglobin H disease in childhood. N Engl J Med. 2011; 364(8):710-8. DOI: 10.1056/NEJMoa1010174. View

11.

Taher A, Musallam K, Saliba A, Graziadei G, Garziadei G, Cappellini M . Hemoglobin level and morbidity in non-transfusion-dependent thalassemia. Blood Cells Mol Dis. 2015; 55(2):108-9. DOI: 10.1016/j.bcmd.2015.05.011. View

12.

Lai K, Huang G, Su L, He Y . The prevalence of thalassemia in mainland China: evidence from epidemiological surveys. Sci Rep. 2017; 7(1):920. PMC: 5430438. DOI: 10.1038/s41598-017-00967-2. View

13.

Chinprateep B, Ratanasit N, Kaolawanich Y, Karaketklang K, Saiviroonporn P, Viprakasit V . Prevalence of left ventricular diastolic dysfunction by cardiac magnetic resonance imaging in thalassemia major patients with normal left ventricular systolic function. BMC Cardiovasc Disord. 2019; 19(1):245. PMC: 6833207. DOI: 10.1186/s12872-019-1235-8. View

14.

Taher A, Musallam K, El-Beshlawy A, Karimi M, Daar S, Belhoul K . Age-related complications in treatment-naïve patients with thalassaemia intermedia. Br J Haematol. 2010; 150(4):486-9. DOI: 10.1111/j.1365-2141.2010.08220.x. View

15.

Ekwattanakit S, Siritanaratkul N, Viprakasit V . A prospective analysis for prevalence of complications in Thai nontransfusion-dependent Hb E/β-thalassemia and α-thalassemia (Hb H disease). Am J Hematol. 2018; 93(5):623-629. DOI: 10.1002/ajh.25046. View

16.

Musallam K, Cappellini M, Daar S, Karimi M, El-Beshlawy A, Graziadei G . Serum ferritin level and morbidity risk in transfusion-independent patients with β-thalassemia intermedia: the ORIENT study. Haematologica. 2014; 99(11):e218-21. PMC: 4222462. DOI: 10.3324/haematol.2013.097220. View

17.

Musallam K, Rivella S, Vichinsky E, Rachmilewitz E . Non-transfusion-dependent thalassemias. Haematologica. 2013; 98(6):833-44. PMC: 3669437. DOI: 10.3324/haematol.2012.066845. View

18.

Yang G, Liu R, Peng P, Long L, Zhang X, Yang W . How early can myocardial iron overload occur in beta thalassemia major?. PLoS One. 2014; 9(1):e85379. PMC: 3899006. DOI: 10.1371/journal.pone.0085379. View

19.

Taher A, Cappellini M, Aydinok Y, Porter J, Karakas Z, Viprakasit V . Optimising iron chelation therapy with deferasirox for non-transfusion-dependent thalassaemia patients: 1-year results from the THETIS study. Blood Cells Mol Dis. 2016; 57:23-9. DOI: 10.1016/j.bcmd.2015.11.002. View

20.

Maakaron J, Cappellini M, Graziadei G, Bou Ayache J, Taher A . Hepatocellular carcinoma in hepatitis-negative patients with thalassemia intermedia: a closer look at the role of siderosis. Ann Hepatol. 2013; 12(1):142-6. View