中文摘要：

目的通过检测骨髓增生异常综合征（MDS）输血依赖患者血清铁代谢平衡相关激素Hepcidin水平及铁负荷指标，探讨Hepcidin联合铁负荷指标在临床诊治MDS患者铁过载中的应用价值。方法采用竞争酶联免疫吸附试验、放射免疫法、比色法分别测定68例MDS输血依赖患者的106份血清、无需输血患者30份血清及60名正常对照者血清中的Hepcidin、血清铁蛋白（SF）及血清铁（SI）水平。结果MDS组中输血〈9U者，Hepcidin水平显著高于对照组[（583±50）μg/L对（175±35）μg/L]，Hepcidin与SF水平呈正相关（r=0．976），与SI水平无相关性（r=0．284）；输血9～24U者，Hepcidin水平与SF水平显著高于对照组[（665±80）μg/L对（175±35）μg/L；（1445±275）μg/L对（112±26）μg/L]，SI水平与对照组无明显差异，Hepcidin与SF或SI水平无相关性；输血〉24U者，Hepcidin水平与SF及SI均高于对照组[（703±64）μg／L对（175±35）μg/L；（2587±352）μg/L对（112±26）μg／L；（20±4）μg/L对（14±4）μmol／L]，Hepcidin水平与SF、SI均呈负相关（r值分别为-0．536和-0．456）。难治性贫血伴有环状铁粒幼细胞（RARS）患者血清Hepcidin水平显著低于难治性贫血伴原始细胞过多（RAEB）患者[（260±40）μg/L对（442±51）μg／L]，且RARS组患者不论输血次数Hepeidin水平均与对照组无差异。结论无论是否输血、输血量多少MDS患者Hepcidin、SF水平均较正常人增高，但随着输血量的增加，Hepcidin水平并不随SF增加而同步增加，以至于输血量〉24U后Hepcidin水平与SF、SI均呈负相关，反映随输血量的增加Hepcidin抑制机体铁吸收的能力下降，最后将出现实质性铁过载。通过动态监测MDS输血依赖患者的Hepcidin水平可以预测机体实质性铁过载的发生。

英文摘要：

Objective To explore the application value of detection of Hepcidin together with indicator of iron overload on clinical diagnosis and treatment of MDS with iron overload by measuring Hepcidin and iron load indices of transfusion dependent myelodysplastic syndrome （ MDS ） patients. Methods Enzymelinked immunosorbent assay（ELISA） , radioimmunoassay and eolorimetry were used to determine the Hepcidin, serum ferritin （SF） and serum iron （SI） levels of 106 serum samples from 68 cases of transfusion dependent MDS patients, 30 serum samples of MDS patients without transfusion and 60 serum samples of controis. Results For MDS group, Hepcidin level in blood transfusion 〈 9 U subgroup was significantly higher than that in control group [ （583 ± 50） μg/L vs （175 ±35 ）μg/L] and there was a strong positive correlation between Hepcidin levels and SF（ r = 0. 976）, but no correlation between Hepcidin and SI（ r = 0. 284） ; Both Hepcidin and SF level in transfusion 9 -24 U subgroup was significantly higher than those in control group [ （ 665± 80 ） μg/L vs （ 175 ± 35 ） μg/L; （ 1445 ± 275 ） μg/L vs （ 112 ± 26 ） μg/L ] ; whereas for SI level, there was no difference between transfusion 9 -24 U subgroup and the control group. Hepeidin did not correlate with SF or SI ; For blood transfusion 〉 24 U group, all of Hepeidin, SF and SI levels were higher than those in control groups [ （703 ±64） μg/L vs （175 ±35） μg/L; （2587 ±352） μg/L vs （ 112 ±26） μg/L; （20 ±4） μg/L vs （ 14 ±4） μmol/L], Hepcidin negatively correlated with SF and SI （ r = -0. 536;r =- 0. 456）. Hepcidin levels of RARS patients were significantly lower than RAEB patients [ （260 ± 40） μg/L vs （442 ±51 ）μg/L] , and there was no significant difference between RARS group and control group regardless of the number of blood transfusion. Conclusion Both Hepcidin and SF levels in MDS patients regardless of transfusion dependent or not, or the numnber of blood transftls