[不同基因型地中海贫血患儿铁代谢及红细胞生成增殖状况]

[Status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia].

作者信息

Mai Hui-Rong, Li Chang-Gang, Wang Ying, Shi Hong-Song, Zhao Wei-Ling, Chen Yun-Sheng, Chen Xiao-Wen

机构信息

Department of Hematology, Shenzhen Children's Hospital, Shenzhen, Guangdong 518026, China.

出版信息

Zhongguo Dang Dai Er Ke Za Zhi. 2010 Aug;12(8):602-4.

PMID:20704788

Abstract

OBJECTIVE

To study the status of iron metabolism and erythropoietic proliferation in children with various genotypes of thalassemia.

METHODS

Serum concentrations of ferritin (SF), transferrin receptor (sTfR) and erythropoietin (EPO) were measured in 158 children with thalassemia. The differences in the concentrations of the three indices among children with different genotypes of thalassemia were compared. The correlations of the hemoglobin level with sereum SF, sTfR and EPO levels were assessed.

RESULTS

Among the 158 children with thalassemia, 52(32.9%) were diagnosed with alpha-thalassemia minor, 27(17.1%) with HbH disease, 59(37.4%) with beta-thalassemia minor, 13(8.2%) with beta-thalassemia major, and 7(4.4%) with combining alpha beta thalassemia. The SF levels in children with HbH disease or beta-thalassemia major were significantly higher than those in the other thalassemia groups (P<0.01). The sTfR levels in children with beta-thalassemia major were the highest when compared with those in the other thalassemia groups (P<0.05). The EPO levels in children with beta-thalassemia major were also the highest when compared with those in the other thalassemia groups (P<0.01). There was a negative correlation between hemoglobin and EPO levels in children with HbH disease (r=-0.656, P<0.01) and beta-thalassemia major (r=-0.641; P<0.05).

CONCLUSIONS

The status of iron metabolism and erythropoietic proliferation is different in children with different genotypes of thalassemia. A combined measurement of SF, sTfR and EPO may reflect the status of erythropoietic proliferation.

摘要

目的

研究不同基因型地中海贫血患儿铁代谢及红细胞生成增殖状况。

方法

检测158例地中海贫血患儿血清铁蛋白（SF）、转铁蛋白受体（sTfR）及促红细胞生成素（EPO）水平，比较不同基因型地中海贫血患儿上述三项指标水平的差异，评估血红蛋白水平与血清SF、sTfR及EPO水平的相关性。

结果

158例地中海贫血患儿中，轻型α地中海贫血52例（32.9%），血红蛋白H病27例（17.1%），轻型β地中海贫血59例（37.4%），重型β地中海贫血13例（8.2%），αβ复合型地中海贫血7例（4.4%）。血红蛋白H病或重型β地中海贫血患儿的SF水平显著高于其他地中海贫血组（P<0.01）。重型β地中海贫血患儿的sTfR水平高于其他地中海贫血组（P<0.05）。重型β地中海贫血患儿的EPO水平也高于其他地中海贫血组（P<0.01）。血红蛋白H病患儿（r=-0.656，P<0.01）及重型β地中海贫血患儿（r=-0.641；P<0.05）的血红蛋白水平与EPO水平呈负相关。