Uaprasert Noppacharn, Rojnuckarin Ponlapat, Bhokaisawan Narudee, Settapiboon Rung, Wacharaprechanont Teera, Amornsiriwat Siriporn, Sutcharitchan Pranee
Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Clin Chim Acta. 2009 May;403(1-2):110-3. doi: 10.1016/j.cca.2009.01.031. Epub 2009 Feb 6.
Serum transferrin receptor (sTfR) measurement is a helpful test for diagnosis of iron deficiency. Increased values are detectable in thalassemia syndromes due to increased erythropoiesis. However, sTfR has never been studied in hemoglobin E (HbE) carriers and their interactions with alpha-thalassemia heterozygotes that are common in Southeast Asia.
We determined sTfR concentrations using a particle enhanced immunoturbidimetric assay in 113 early pregnancies without iron deficiency.
Patients were genotypically classified into 6 groups: 23 normal (mean sTfR+/-SD mg/l, 0.94+/-0.22), 14 alpha(+)-thalassemia heterozygotes (1.06+/-0.45), 21 alpha(0)-thalassemia heterozygotes (1.31+/-0.35), 30 HbE heterozygotes (1.11+/-0.26), 13 HbE heterozygotes with alpha(+)-thalassemia heterozygotes (1.09+/-0.32), and 12 HbE heterozygotes with alpha(0)-thalassemia heterozygotes (1.16+/-0.27). sTfR concentrations in all thalassemic groups were higher than controls, and significantly correlated with high red cell count, low MCV and MCH (p<0.001). When alpha(0)- or alpha(+)-thalassemia combined with HbE, sTfR concentrations were declined compared with alpha(0)-thalassemia or hemoglobin E, respectively, suggesting more balances in alpha- and beta-globin chain production.
Mildly increased erythropoiesis represented by increased sTfR concentrations in alpha-thalassemia and HbE heterozygotes and illustrated alpha- and beta-thalassemic gene interaction. These findings warrant further investigations on sTfR in diagnosis of iron deficiency in thalassemia carriers.
血清转铁蛋白受体(sTfR)检测对缺铁性贫血的诊断有一定帮助。由于红细胞生成增加,地中海贫血综合征患者的sTfR值会升高。然而,此前从未对血红蛋白E(HbE)携带者及其与东南亚地区常见的α地中海贫血杂合子之间的相互作用进行过研究。
我们采用颗粒增强免疫比浊法测定了113例无缺铁情况的早孕患者的sTfR浓度。
患者按基因型分为6组:23例正常者(平均sTfR±标准差mg/l,0.94±0.22),14例α(+)地中海贫血杂合子(1.06±0.45),21例α(0)地中海贫血杂合子(1.31±0.35),30例HbE杂合子(1.11±0.26),13例HbE杂合子合并α(+)地中海贫血杂合子(1.09±0.32),12例HbE杂合子合并α(0)地中海贫血杂合子(1.16±0.27)。所有地中海贫血组的sTfR浓度均高于对照组,且与高红细胞计数、低平均红细胞体积(MCV)和平均红细胞血红蛋白含量(MCH)显著相关(p<0.001)。当α(0)或α(+)地中海贫血与HbE合并时,sTfR浓度分别比单独的α(0)地中海贫血或血红蛋白E降低,提示α和β珠蛋白链的产生更加平衡。
α地中海贫血和HbE杂合子中sTfR浓度升高代表红细胞生成轻度增加,显示了α和β地中海贫血基因的相互作用。这些发现为进一步研究sTfR在诊断地中海贫血携带者缺铁情况中的应用提供了依据。