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采用现代螯合疗法治疗的成年重型β地中海贫血患者肝脏铁负荷的降低

Reduction of Liver Iron Load in Adult Patients with β-Thalassemia Major Treated with Modern Chelation Modalities.

作者信息

Georgiev Pencho G, Sapunarova Katya G, Goranova-Marinova Veselina S, Goranov Stefan E

机构信息

Hematology Section, First Department of Internal Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria.

出版信息

Folia Med (Plovdiv). 2020 Jun 30;62(2):265-270. doi: 10.3897/folmed.62.e39518.

DOI:10.3897/folmed.62.e39518
PMID:32666765
Abstract

BACKGROUND

Management of beta-thalassemia major (TM) requires life-long hemotransfusions leading to iron overload. Iron elimination is enhanced by the use of modern chelators.

AIM

To assess the effect of modern chelation therapy by dynamics of serum ferritin concentration and liver MRI T2*.

PATIENTS AND METHODS

Forty-six patients with TM (male to female ratio =1:1, mean age 33.2±10.9 years) were prospectively studied between 2011 and 2014. Twenty-one patients (45.7%) were treated with deferasirox, 17 (37%) - with deferiprone, and 8 (17.3%) - with deferiprone in combination with deferoxamine. Ferritin was measured by ELISA. MRI T2* was assessed by Siemens Magnetom Avanto 1.5T. The patients were allocated into 3 groups based on their initial ferritin level and liver MRI T2*. Statistical analysis was performed using SPSS v. 18 for Windows. Data were analysed by descriptive analysis, analysis of variance and correlative analysis, means were compared using t-test and one-way ANOVA.

RESULTS

In 2011, 9 (19.5%) patients had normal liver MRI T2*; in 2014 they were 17 (37%). The patients with mild grade liver siderosis were 12 (26%) in 2011, and in 2014 they were 14 (30.4%). In 2011, the patients with moderate liver siderosis were 14 (30.4%), and in 2014 - 12 (26.0%). Eleven patients (23.9%) had severe liver siderosis in 2011 and only two patients (4.0%) were diagnosed with the condition in 2014.

CONCLUSION

A reduction of iron overload was found in all studied groups. This positive effect is attributed to the use of modern chelators and the ease of access to accurate monitoring.

摘要

背景

重型β地中海贫血(TM)的管理需要终身输血,这会导致铁过载。现代螯合剂的使用可增强铁的清除。

目的

通过血清铁蛋白浓度动态变化和肝脏MRI T2*评估现代螯合疗法的效果。

患者与方法

2011年至2014年对46例TM患者(男女比例为1:1,平均年龄33.2±10.9岁)进行前瞻性研究。21例患者(45.7%)接受地拉罗司治疗,17例(37%)接受去铁酮治疗,8例(17.3%)接受去铁酮联合去铁胺治疗。铁蛋白通过酶联免疫吸附测定法测量。MRI T2由西门子Magnetom Avanto 1.5T评估。根据患者初始铁蛋白水平和肝脏MRI T2将其分为3组。使用Windows版SPSS v. 18进行统计分析。数据通过描述性分析、方差分析和相关性分析进行分析,均值比较采用t检验和单因素方差分析。

结果

2011年,9例(19.5%)患者肝脏MRI T2*正常;2014年为17例(37%)。2011年轻度肝脏铁沉着症患者为12例(26%),2014年为14例(30.4%)。2011年中度肝脏铁沉着症患者为14例(30.4%),2014年为12例(26.0%)。2011年11例患者(23.9%)有严重肝脏铁沉着症,2014年仅2例患者(4.0%)被诊断为此病。

结论

在所有研究组中均发现铁过载有所减轻。这种积极效果归因于现代螯合剂的使用以及准确监测的便捷性。

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