Abstract：OBJECTIVE: To study the status of iron deposition in patients with β-thalassemia intermedia and major in mainland China. METHODS: The status of transfusion and chelation was examined in 39 patients with β-thalassemia intermedia or major. Serum ferritin levels were measured. MRI T2* technique was used to detect cardiac and hepatic iron deposition. RESULTS: Serum ferritin levels ranged from the minimum of 1500 ng/mL up to a maximum of 11491 ng/mL. From liver MRI T2* measurement, 15 cases had severe hepatic iron deposition (38%) and moderate deposition was found in 15 cases (38%), mild in 7 cases (18%), and normal in 2 cases (5%). Heart MRI T2* showed severe heart iron deposition in 7 cases (18%), mild in 5 cases (13%), and normal in 27 cases (69%). One case had cardiac arrhythmia. Four cases were over 20 years of age, and presented with gonadal function hypoplasia. The majority of patients did not receive regular transfusion and they had delayed, suboptimal chelation due to financial problems. Serum ferritin level was closely related with timing and dosage of chelation. CONCLUSIONS: In patients with β-thalassemia who do not receive early regular transfusion and iron chelation therapy, iron deposition may occur at an early age. Important organs and tissue functional lesions and related complications also result. Relevant agencies and family members should be aware of this trend and develop appropriate strategies to improve the medical condition and quality of life of patients with this disorder.
LI Chang-Gang,LIU Si-Xi,MAI Hui-Rong et al. Evaluation of heart and liver iron deposition status in patients with β- thalassemia intermedia and major with MRI T2* technique[J]. CJCP, 2012, 14(2): 110-113.
［1］Li CG, Li CF, Li Q, Li M. Thalassemia incidence and treatment in China with special reference to Shenzhen City and Guangdong province［J］. Hemoglobin, 2009, 33(5): 296-303.
［2］Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E. Thalassemia ［J］. Hematology Am Soc Hematol Educ Program, 2004: 14-34.
［3］Beutler E, Hoffbrand AV, Cook JD. Iron deficiency and overload［J］. Hematology Am Soc Hematol Educ Program, 2003: 40-61.
［4］Au WY, Lam WW, Chu WW, Yuen HL, Ling AS, Li RC, et al. A cross-sectional magnetic resonance imaging assessment of organ specific hemosiderosis in 180 thalassemia major patients in Hong Kong［J］. Haematologica, 2008, 93(5): 784-786.
［6］Borgna-Pignatti C, Rugolotto S, De Stefano P, Piga A, Di Gregorio F, Gamberini MR, et al. Survival and disease complications in thalassemia major［J］. Ann N Y Acad Sci. 1998, 30(850): 227-231.
［7］Gabutti V, Piga A. Results of long-term iron-chelating therapy［J］. Acta Haematol, 1996, 95(1): 26-36.
［9］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［J］. Haematologica, 2004, 89(10): 1187-1193.
［10］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［J］. J Cardiovasc Magn Reson, 2008, 10: 42.
［11］Pennell DJ. Berdoukas V, Karagiorga M, Ladis V, Piga A, Aessopos A, et al. Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis ［J］. Blood, 2006, 107(9): 3738-3744.
［12］Tanner MA, Galanello R, Dessi C, Smith GC, Westwood MA, Agus A, et al. 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［J］. Circulation, 2007, 115(14): 1876-1884.
