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β地中海贫血的治疗

Treatment of beta-thalassemia.

作者信息

Giardini C

机构信息

Divisione di Ematologia e Centro Trapianto di Midollo Osseo Azienda Ospedaliera S. Salvatore, Pesaro, Italy.

出版信息

Curr Opin Hematol. 1997 Mar;4(2):79-87. doi: 10.1097/00062752-199704020-00002.

DOI:10.1097/00062752-199704020-00002
PMID:9107523
Abstract

The striking improvement in the life expectancy of patients with homozygous beta-thalassemia observed over the past three decades is mainly due to the institution of adequate transfusion regimens and effective iron chelation therapy with nightly subcutaneous desferrioxamine. The prognosis appears particularly favorable for children with thalassemia born since these methods have become widely available. The first successful "cure" of beta-thalassemia was achieved in 1981 after bone marrow transplantation. Recent advances in transfusion techniques, pharmacology, molecular genetics, transplant immunology, and clinical practice today offer considerable promise in further advancing our knowledge and treatment of this disease.

摘要

在过去三十年中观察到的纯合子β地中海贫血患者预期寿命的显著改善,主要归功于适当输血方案的制定以及每晚皮下注射去铁胺进行的有效铁螯合治疗。自从这些方法广泛应用以来,对于患有地中海贫血的儿童来说,预后似乎特别良好。1981年骨髓移植后首次成功“治愈”了β地中海贫血。如今输血技术、药理学、分子遗传学、移植免疫学和临床实践方面的最新进展,为进一步增进我们对这种疾病的认识和治疗带来了很大希望。

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Treatment of beta-thalassemia.β地中海贫血的治疗
Curr Opin Hematol. 1997 Mar;4(2):79-87. doi: 10.1097/00062752-199704020-00002.
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[Bone marrow transplantation in thalassemia].[地中海贫血的骨髓移植]
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引用本文的文献

1
Beta-Thalassemia in Adulthood Previously Suspected as Treatment-Resistant Iron Deficiency Anemia: A Case Report.成年期β地中海贫血曾被怀疑为难治性缺铁性贫血:一例报告
Cureus. 2024 May 14;16(5):e60275. doi: 10.7759/cureus.60275. eCollection 2024 May.
2
2003 William Allan Award address. The Thalassemias: the role of molecular genetics in an evolving global health problem.2003年威廉·艾伦奖演讲。地中海贫血:分子遗传学在一个不断演变的全球健康问题中的作用。
Am J Hum Genet. 2004 Mar;74(3):385-92. doi: 10.1086/381402.
3
Estimates of the effect on hepatic iron of oral deferiprone compared with subcutaneous desferrioxamine for treatment of iron overload in thalassemia major: a systematic review.
与皮下注射去铁胺相比,口服去铁酮治疗重型地中海贫血铁过载对肝脏铁影响的评估:一项系统评价
BMC Blood Disord. 2002 Nov 20;2(1):4. doi: 10.1186/1471-2326-2-4.