不同类型地中海贫血的血清可溶性转铁蛋白受体水平研究

A discussion on clinical significance for the detection of serum soluble transferring receptor in children with thalassemia

目的:探讨血清可溶性转铁蛋白受体检测在不同类型地中海贫血(地贫)儿童中的临床价值。方法:对选取的9例静止型,56例标准型α地贫,26例H病,40例β地贫杂合子,56例β地贫双重杂合子(或纯合子),45例缺铁性贫血(IDA)患儿以及70例健康儿童血清sTfR以及各种铁指标进行检测和统计学分析。结果:sTfR水平在β地贫双重杂合子组最高,明显高于其余各组(均P<0.01),H病组与IDA组水平接近(P>0.05),且均分别高于静止型α地贫、标准型α地贫和β地贫杂合子组(P<0.01);而静止型α地贫、标准型α地贫和β地贫杂合子3组间差异无统计学意义(P>0.05)。结论:①不同基因型的地贫患儿存在着不同程度的sTfR水平升高,地贫高发区sTfR水平升高但不伴缺铁的小细胞性贫血患儿,应着重加强对地贫的诊断。②sTfR水平是辅助诊断β地贫双重杂合子、以及β地贫双重杂合子与其他类型地贫、IDA鉴别诊断的有用指标,但对于区分不同基因型的α地贫、β地贫杂合子意义不大。

Objective：To explore the clinical significance for the detection of serum soluble transferring receptor （sTfR） in children with thalassemia （thal） heterozygotes.Method：302 samples with the Han nationality children were divided into 7 groups including silent α thal,standard α thal,HbH disease,β＋ thal,β 0 thal,iron deficiency anaemia （IDA） and healthy children group. Every groups detected serum sTfR and other parameters of iron,then analyzed the data.Result：The sTfR level of β 0 thal group was obviously higher than the other groups （P0.01）,that of the HbH disease group was similar to the IDA group （P0.05）. HbH and IDA groups were higher than the silent α thal group,the standard α thal group and the β ＋ thal group （P0.01）. There was no distinct difference among the silent α thal,standard α thal and the β ＋ thal group （P0.05）.Conclusion：①Different genotype thalassemia has different sTfR levels. Samicrocytic anaemia children from high frequency thalassemia region with high sTfR level,but without iron lack should be considered firstly as thalassemia. ②sTfR leve is a useful parameter for β 0 thal assistant diagnose,and distinguish diagnose of β 0 thal to other types of thalassemia and IDA. However,it is not available in distinguishing different genotype of α thal and β thal.