The consequence of regular blood transfusion in patients with thalassemia major (TM) is iron overload. Herein, we report the long-term impact of chelation on liver iron concentration (LIC) and cardiac T2* MR in patients with TM. This is a retrospective cohort study over 10 years of adolescents and adults with TM aged at least 10 years who had their first cardiac T2* MR between September 2006 and February 2007. One-year chelation therapy was considered the unit of analysis. A total of 99 patients were included in this study with a median age of 18 years. The median cardiac T2* MR and LIC at baseline were 19 ms and 11.6 mg/g dw, respectively. During follow-up, 18 patients died and six underwent successful bone marrow transplantation. Factors associated with decreased survival were older age (HR 1.12, p = 0.014) and high risk cardiac T2* (HR 8.04, p = 0.004). The median cardiac T2* and LIC significantly improved over the 10-year follow-up period (p = 0.000011 and 0.00072, respectively). In conclusion, this long-term “real-life” study confirms that low cardiac T2* adversely impacts the overall survival in patients with TM. Higher baseline LIC predicts a larger reduction in LIC, and lower baseline cardiac T2* predicts a larger improvement in T2*.

重度地中海贫血（TM）患者定期输血的结果是铁超负荷。在本文中，我们报道了螯合剂对TM患者肝铁浓度（LIC）和心脏T2 * MR的长期影响。这是一项回顾性队列研究，研究对象为2006年9月至2007年2月间首次患有心脏T2 * MR的10岁以下青少年和成年TM成年人。分析的单位是一年螯合疗法。这项研究共纳入99名患者，中位年龄为18岁。基线时的中位心脏T2 * MR和LIC分别为19 ms和11.6 mg / g dw。在随访过程中，有18例患者死亡，其中6例接受了成功的骨髓移植。与存活率降低相关的因素是年龄较大（HR 1.12，p = 0.014）和高危心脏T2 *（HR 8.04，p  = 0.004）。在10年的随访期内，中位心脏T2 *和LIC显着改善（ 分别为p = 0.000011和0.00072）。总之，这项长期的“现实生活”研究证实，低心脏T2 *对TM患者的总体生存产生不利影响。基线较高的LIC预测LIC降低更大，而基线较低的心脏T2 *预测T2 *改善更大。