伊朗德黑兰 34030 名健康成年人中血红蛋白病的患病率。

Prevalence of haemoglobinopathies in 34,030 healthy adults in Tehran, Iran.

机构信息

Pathology Department, Children Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran.

出版信息

J Clin Pathol. 2009 Oct;62(10):924-5. doi: 10.1136/jcp.2009.064568.

DOI:10.1136/jcp.2009.064568

PMID:19783722

Abstract

BACKGROUND

Haemoglobinopathies are a group of inherited disorders of haemoglobin synthesis. Their frequency varies considerably with geographic location and ethnic group.

AIMS

To establish the prevalence of haemoglobinopathies in a mainly healthy Iranian population.

METHODS

All files of the haematology unit of the Boghrat laboratory over a period of 10 years (1998-2007) were analysed in relation to the age, sex, full blood count, haemoglobin electrophoresis results, high performance liquid chromatography (HPLC) findings, and iron profile of healthy subjects referred for consultation before marriage. Other tests were performed if indicated.

RESULTS

There were 34,030 files; 13,432 (39.5%) were for men, and 20,567 (60.4%) for women. 0.74% of subjects (255) showed a haemoglobinopathy. The distribution of variant haemoglobins in these 255 subjects was: Hb D 75.67% (193 cases), Hb S 4.7%, Hb E 3.13%, Hb O-Arab 1.96% and Hb Lepore 0.39%. A fast haemoglobin was found in 4.71% of subjects. Of the subjects tested, 13.2% (4478) had beta-thalassaemia minor, 0.2% (52) beta-thalassaemia intermedia and 0.1% (30) beta-thalassaemia major.

CONCLUSIONS

In comparison with other parts of world, there is a different pattern of haemoglobinopathy with a high prevalence of Hb D, which appears to be Hb D Iran.

摘要

背景

血红蛋白病是一组遗传性血红蛋白合成紊乱。其频率因地理位置和种族群体而异。

目的

确定主要为健康伊朗人群中血红蛋白病的流行情况。

方法

分析了 Boghrat 实验室血液学部门 10 年来（1998-2007 年）的所有档案，这些档案与健康受试者在婚前咨询时的年龄、性别、全血细胞计数、血红蛋白电泳结果、高效液相色谱（HPLC）结果和铁谱有关。如有必要，还进行了其他测试。

结果

共有 34030 份档案；其中 13432 份（39.5%）为男性，20567 份（60.4%）为女性。0.74%（255 人）的受试者显示血红蛋白病。这 255 名受试者中变异血红蛋白的分布为：Hb D 75.67%（193 例），Hb S 4.7%，Hb E 3.13%，Hb O-Arab 1.96%和 Hb Lepore 0.39%。在 4.71%的受试者中发现了快速血红蛋白。在接受测试的受试者中，13.2%（4478 人）有轻度β地中海贫血，0.2%（52 人）有中间型β地中海贫血，0.1%（30 人）有重型β地中海贫血。