Aydinok Yesim
Ege University Children's Hospital, Department of Pediatric Hematology, Ege University Hospital Blood Bank, 35100 Bornova, Izmir, Turkey.
Hematology. 2012 Apr;17 Suppl 1:S28-31. doi: 10.1179/102453312X13336169155295.
Thalassemia is the most common form of inherited anemia worldwide. The World Health Organization reports suggest that about 60,000 infants are born with a major thalassemia every year. Although individuals originating from the tropical belt are most at risk, it is a growing global health problem due to extensive population migrations. Despite important advances on curative approaches such as stem cell transplantation and promising results of gene therapy, blood transfusions and iron chelation still remain as cornerstones of disease management. The purpose of this article is to focus on mainly the clinical aspects and management of beta-thalassemia major.
地中海贫血是全球最常见的遗传性贫血形式。世界卫生组织的报告显示,每年约有6万名婴儿出生时患有重度地中海贫血。尽管来自热带地区的人风险最高,但由于大规模人口迁移,这已成为一个日益严重的全球健康问题。尽管在干细胞移植等治疗方法方面取得了重要进展,基因治疗也有了令人鼓舞的结果,但输血和铁螯合疗法仍然是疾病管理的基石。本文的目的主要是关注重型β地中海贫血的临床方面和管理。