Retrospective nationwide survey of Japanese patients with transfusion-dependent MDS and aplastic anemia highlights the negative impact of iron overload on morbidity/mortality
Retrospective nationwide survey of Japanese patients with transfusion-dependent MDS and aplastic anemia highlights the negative impact of iron overload on morbidity/mortality
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DOI：
10.1111/j.1600-0609.2007.00842.x
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发表时间：
2007-06-01
影响因子：
3.1
通讯作者：
Ozawa, Keiya
中科院分区：
文献类型：
--
作者：
Takatoku, Masaaki;Uchiyama, Takashi;Ozawa, Keiya
目的：骨髓增生异常综合征(MDS)和再生障碍性贫血(AA)是日本最常见的需要输血治疗的贫血。这项回顾性调查调查了铁超载、螯合实践和这些疾病患者的发病率/死亡率之间的关系。方法：对输血依赖患者的病史在输血开始时、螯合开始时和研究结束时进行评估。结果：收集了292名患有MDS、再生障碍性贫血、纯红细胞再生障碍性贫血、骨髓纤维化和其他疾病的患者的数据。患者在前一年平均接受了61.5个红细胞单位。不到一半(43%)的患者以前接受过去铁胺(DFO)治疗。只有8.6%的人接受了每天或持续的DFO。总共报告了75例死亡，24.0例和6.7%的病例出现了心脏和肝脏衰竭。在这些人中，97%的人铁蛋白水平为1000 ng/ml。心、肝功能异常者分别为21.9%(14/)和84.6%(11/13)。与DFO的有效螯合可改善血清铁蛋白、肝酶和空腹血糖。结论：严重铁负荷患者病死率较高，肝、心功能不全是主要原因。每日/持续螯合疗法能有效降低铁负荷，改善器官功能。一旦血清铁蛋白水平超过1000纳克/毫升，就应该开始螯合治疗。
Objective: Myelodysplastic syndromes (MDS) and aplastic anemia (AA) are the most common anemias that require transfusion therapy in Japan. This retrospective survey investigated relationships between iron overload, chelation practices, and morbidity/mortality in patients with these diseases. Method: Medical histories of transfusion-dependent patients were assessed at transfusion onset, chelation onset, and study end. Results: Data were collected from 292 patients with MDS, AA, pure red cell aplasia, myelofibrosis, and other conditions. Patients received a mean of 61.5 red blood cell units during the previous year. Fewer than half (43%) of patients had previously received deferoxamine (DFO) therapy. Only 8.6% received daily/continuous DFO. In all, 75 deaths were reported, with cardiac and liver failure noted in 24.0 and 6.7% of cases. Of these, 97% had ferritin levels > 1000 ng/mL. Abnormal cardiac and liver function was observed in 21.9% (14/64) and 84.6% (11/13) of all patients assessed. Effective chelation with DFO resulted in improved serum ferritin, liver enzymes, and fasting blood sugar. Conclusion: Mortality is higher in heavily iron-overloaded patients, with liver and cardiac dysfunction being the primary cause. Daily/continuous chelation therapy was effective at reducing iron burden and improving organ function. Chelation therapy should be initiated once serum ferritin levels exceed 1000 ng/mL.