血红蛋白H(HbH)病患者体内铁负荷的研究

Study of the levels of serum transferrin receptors (sTfR) and iron overload in hemoglobin H patients

目的检测血红蛋白H病患者体内血清转铁蛋白受体(sTfR)及血清铁蛋白(SF)的浓度,了解HbH病患者体内的铁负荷状况,并分成缺失型和非缺失型两组,通过对sTfR及其复合参数sTfR/1gSF进行分析比较,重点了解缺失型和非缺失型HbH病患者体内铁负荷状况。方法采用酶联免疫吸附反应(ELISA)分别检测26例缺失型(HbH)及24例非缺失型(HbH-CS)患者及30例健康对照者血浆中的sTfR及SF的浓度,通过计算分别得出两组的sTfR/1gSF值;以血清铁蛋白作为标准对sTfR/1gSF、sTfR这两个参数在诊断HbH病患者体内是否存在铁超负荷状况时的价值进行分析。结果在缺失型和非缺失型(HbH-CS)分组中,SF值的比较,两组值均高于对照组,且非缺失型增高较缺失型明显,有统计学意义:sTfR值缺失型和非缺失型两组值均低于对照组,有统计学意义,但两组间sTfR值比较无统计学意义;缺失型与非缺失型组的sTfR/1gSF均数与标准差经两样本t检验,差异有统计学意义(P<0.01),非缺失型组低于缺失型组。结论HbH病患者sTfR水平降低,结合SF结果联合判断,提示有铁超负荷的存在,非缺失型的患者较缺失型患者更易发生铁趋负荷。

Objective To measure the levels of serum transferrin receptors （sTIR） in patients with hemoglobin H disease for investigation of iron metabolism （ sideropenic or iron overload） which guide to proper modalities of treatment. In this study, we distributed 2 groups according to its types; deletion types of hemoglobin H disease （HbH） and non-deletion types of hemoglobin H disease （HbH-CS）. Iron overload condition in HbH patients are analysed by comparing the parameters of sTIR and sTfR/1gSF. Methods Peripheral blood specimens were obtained from 26 cases of deletion types of hemoglobin H disease patients, 24 cases of non-deletion types of hemoglobin H disease （HbH-CS） and 30 cases of normal cases for controls. The levels of sTIR and SF were examined by Enzyme-linked immunosorbent assay （ELISA）. Results The levels of SF in both HbH patient group was higher than the control group（P 〈0. 01）, The levels of SF in HbH-CS patients has significantly higher than HbH patients （P 〈 0. 01 ）. The levels of sTfR in both HbH patient group has siguificandy lower than the control group （P 〈 0. 01 ）, but there was no statistical significance between two groups; The sTfR/1gSF value in HbH-CS patients has significantly lower than HbH patients （P 〈 0. 01 ）. Conclusions The levels of sTIR in both HbH patient group was lower than the control group according to the value of SF, iron overload was found in both groups, in non-deletion types of hemoglobin H disease （HbH-CS） there was higher risk of iron overloads than HbH patients.