广西地中海贫血112例临床分析  

Clinical analysis on 112 cases of β-Thalassemia in Guangxi Province

作　　者：尹芳[1] 程鹏[1] 张新华[2] 尹晓林[2] 邓东红[1] 

机构地区：[1]广西医科大学第一附属医院,第一临床医学院血液内科,广西南宁530021 [2]中国人民解放军三〇三医院血液内科,广西南宁530021

出　　处：《蛇志》2014年第2期159-162,共4页Journal of Snake

基　　金：广西医疗卫生重点科研课题(重2012077)

摘　　要：目的通过对广西112例地中海贫血病例的回顾性分析,了解地中海贫血患者的临床特点、治疗现状和并发症。方法用回顾性分析方法对广西112例地中海贫血患者(α中间型16例,β中间型30例,β重型66例)的临床特点、治疗现状和并发症进行分析。结果 (1)52例β重型地贫患者和所有需长时间频繁输血的中间型地贫患者输血前Hb<90g/L。(2)不规范祛铁治疗的β中间型和β重型患者分别为84.0%和89.8%。(3)112例患者中,74例(67.9%)存在铁过载,2例α中间型,19例β中间型,53例β重型。(4)2例β中间型和1例β重型患者曾发生心力衰竭;2例β重型患者有继发性糖尿病;17例β中间型和46例β重型患者身材矮小,身材矮小与铁蛋白、输血前血红蛋白量相关(r=0.336,P<0.001;r=-0.243,P=0.011)。结论本组β中间型地贫和β重型地贫患者输血和去铁治疗不规范,铁过载严重,身材矮小、心力衰竭等并发症发生率高。Objective Through the retrospective analysis of 112 cases of thalassemia in Guangxi province ,explore the clinical features ,treatment status quo and complication in patients with thalassemia . Methods Analysis of the clinical characteristics ,treatment and complication of 112 thalassemia patients（16α-thalassemia intermediate ,30β-thalassemia intermediate ,66β-thalassemia major） in Guangxi with a retrospective analyzed method . Results （1）52β-thalassemia major patients and all long time frequent blood transfusions patients of β-thalassemia interme-dia with pre-transfusion Hb〈90 g/L .（2）Iron chelation therapy was used irregularly in 84% patients of β-thalasse-mia intermediate and 89.8% patients of β-thalassemia major .（3）In 112 thalassemia patients ,74 patients （67.9% ） with iron overload ,including two α-thalassemia intermediate patients ,19 β-thalassemia intermediate patients ,53 β-thalassemia major patients .（4）Two β-thalassemia intermediate patients and one β-thalassemia major patient with severe heart failure ;two β-thalassemia major patients with secondary diabetes mellitus ;17β-thalassemia intermedi-ate patients and 46β-thalassemia major patients with severe short stature ,short stature was correlated with levels of ferritin and pre-transfusion Hb （r=0.336 ,P〈0.001 ;r= -0.243 ,P=0.011） . Conclusion Our data showed that ,blood transfusion and iron chelation therapy were not standardized in the β-thalassemia intermedia and β-thalassemia major patients ,iron overload severely ,a high incidence of complications such as heart failure ,diabetes mellitus and short stature .

关 键 词：地中海贫血 临床特点 治疗现状 

分 类 号：R714.254[医药卫生—妇产科学]