中文摘要：

目的研究骨髓增生异常综合征（MDS）患者铁代谢参数和临床特征。方法对2015年6月至2016年3月间初诊未经祛铁治疗的94例MDS患者的铁代谢参数和临床特征进行回顾性调查。结果94例初治MDS患者中，71例（75.53%）贫血，56例（59.57%）存在输血依赖，52例（55.32%）存在铁过载。IPSS低危／中危-1较中危-2/高危铁过载多见（P〈0.01）。铁过载组和非铁过载组比较，血清铁（SI）升高[36.5（8.5-64.7）μmol/L对25.2（3.7-45.3）μmol/L，P〈0.01]，转铁蛋白饱和度（TSAT）降低[43.5%（12.2%-77.2%）对53.4%（14.8%-97.5%），P〈0.01]，不稳定细胞铁（LCI）及细胞内活性氧簇（ROS）差异无统计学意义。靶器官（心、肝、胰岛等）功能临床资料比较中，铁过载组ALT及B型钠尿肽前体分别为25（3-158）U/L及190（6-4 281）ng/L，均明显高于非铁过载组的16（5-80）U/L及84（12-2 275）ng/L（P=0.03及P=0.05）。难治性贫血伴环状铁粒幼红细胞（RARS）患者与非RARS患者铁代谢参数差异均无统计学意义（P〉0.05），输血依赖程度和输血量显著影响SI、TSAT及SF（P值均〈0.01），但LCI及ROS组间差异无统计学意义。无输血依赖、非铁过载的38例患者中，骨髓红系病态造血、幼红细胞比例增高组LCI水平显著增高。结论初治MDS患者贫血及输血依赖现象较为常见，输血频率和输血量显著影响铁代谢参数。而无输血依赖的非铁过载患者骨髓红系病态造血、幼红细胞比例增高影响LCI水平，LCI可以作为衡量铁过载和铁分布重要的早期指标。

英文摘要：

ObjectiveTo investigate the abnormalities of iron metabolism parameters, the prevalence and risk factors of iron overload and clinical characteristics of patients with myelodysplastic syndromes（MDS）.MethodsRetrospective investigation was used to observe abnormal iron metabolism parameters and clinical characteristics of newly diagnosed 94 MDS patients in our center from June 2015 to March 2016.ResultsOf 94 patients, 71（75.53%）had a hemoglobin level of less than 100 g/L at diagnosis. Iron overload was observed in 52（55.32%）of 94 MDS patients, in which a higher prevalence of iron overload was observed in low risk groups（IPSS low/Int-1 risk groups）than higher risk groups（Int-2/high risk groups）. Higher levels of serum iron（SI）[36.5（8.5-64.7）mmol/L vs 25.2（3.7-45.3）mmol/L, P〈0.01], transferrin saturation（TSAT）[43.5（12.2-77.2）% vs 53.4（14.8-97.5）%, P 〈0.01]and serum ferritin（SF）were observed in iron overload group. No differences of labile cellular iron（LCI）and reactive oxygen species（ROS）were observed between two groups（P=0.88, P=0.06）. As the results of clinical complication of iron overload, alanine aminotransferase（ALT）[25（3-158）U/L vs 16（5-80）U/L, P=0.03]and type B natriuretic peptide precursor（proBNP）[190（6-4281）ng/L vs 84（12-2 275）ng/L, P= 0.05]levels were increased in iron overload group. There was no significant difference in iron metabolism parameters between patients with refractory anemia（RARS）and non RARS patients（P〉0.05）. Both frequency and volume of RBC transfusion had a significant effect on all iron metabolism parameters（SI, TSAT and SF）（P 〈0.01）except LCI and ROS. Excluded the patients with history of blood transfusion and SF levels over 1 000 μg/L, higher levels of LCI were mainly observed in dysplastic erythropoiesis and increased bone marrow erythroblasts ratio groups（P〈0.01, P〈0.05）.ConclusionThe main cause of iron overload in MDS is chronic transfusion therapy. Both frequency and