{"title":"铁超载:β-地中海贫血症的致命弱点。","authors":"","doi":"10.1016/j.tracli.2024.06.001","DOIUrl":null,"url":null,"abstract":"<div><p>Systematic transfusions coupled with iron chelation therapy have substantially improved the life expectancy of thalassemia patients in developed nations. As the human organism does not have a protective mechanism to remove excess iron, iron overload is a significant concern in thalassemia, leading to organ damage, especially in the heart and liver. Thus, iron chelation therapy is crucial to prevent or reverse organ iron overload. There are three widely used iron chelators, either as monotherapy or in combination. The choice of iron chelator depends on several factors, including local guidelines, drug availability, and the individual clinical scenario. Despite treatment advancements, challenges persist, especially in resource-limited settings, highlighting the need for improved global healthcare access. This review discusses clinical management, current treatments, and future directions for thalassemia, focusing on iron overload and its complications. Furthermore, it underscores the progress in transforming thalassemia into a manageable chronic condition and the potential of novel therapies to further enhance patient outcomes.</p></div>","PeriodicalId":23262,"journal":{"name":"Transfusion Clinique et Biologique","volume":"31 3","pages":"Pages 167-173"},"PeriodicalIF":1.4000,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Iron overload: The achilles heel of β-thalassemia\",\"authors\":\"\",\"doi\":\"10.1016/j.tracli.2024.06.001\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><p>Systematic transfusions coupled with iron chelation therapy have substantially improved the life expectancy of thalassemia patients in developed nations. As the human organism does not have a protective mechanism to remove excess iron, iron overload is a significant concern in thalassemia, leading to organ damage, especially in the heart and liver. Thus, iron chelation therapy is crucial to prevent or reverse organ iron overload. There are three widely used iron chelators, either as monotherapy or in combination. The choice of iron chelator depends on several factors, including local guidelines, drug availability, and the individual clinical scenario. Despite treatment advancements, challenges persist, especially in resource-limited settings, highlighting the need for improved global healthcare access. This review discusses clinical management, current treatments, and future directions for thalassemia, focusing on iron overload and its complications. Furthermore, it underscores the progress in transforming thalassemia into a manageable chronic condition and the potential of novel therapies to further enhance patient outcomes.</p></div>\",\"PeriodicalId\":23262,\"journal\":{\"name\":\"Transfusion Clinique et Biologique\",\"volume\":\"31 3\",\"pages\":\"Pages 167-173\"},\"PeriodicalIF\":1.4000,\"publicationDate\":\"2024-06-06\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Transfusion Clinique et Biologique\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S1246782024000636\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Transfusion Clinique et Biologique","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1246782024000636","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
Systematic transfusions coupled with iron chelation therapy have substantially improved the life expectancy of thalassemia patients in developed nations. As the human organism does not have a protective mechanism to remove excess iron, iron overload is a significant concern in thalassemia, leading to organ damage, especially in the heart and liver. Thus, iron chelation therapy is crucial to prevent or reverse organ iron overload. There are three widely used iron chelators, either as monotherapy or in combination. The choice of iron chelator depends on several factors, including local guidelines, drug availability, and the individual clinical scenario. Despite treatment advancements, challenges persist, especially in resource-limited settings, highlighting the need for improved global healthcare access. This review discusses clinical management, current treatments, and future directions for thalassemia, focusing on iron overload and its complications. Furthermore, it underscores the progress in transforming thalassemia into a manageable chronic condition and the potential of novel therapies to further enhance patient outcomes.
期刊介绍:
Transfusion Clinique et Biologique, the official journal of the French Society of Blood Transfusion (SFTS):
- an aid to training, at a European level
- the only French journal indexed in the hematology and immunology sections of Current Contents
Transfusion Clinique et Biologique spans fundamental research and everyday practice, with articles coming from both sides. Articles, reviews, case reports, letters to the editor and editorials are published in 4 editions a year, in French or in English, covering all scientific and medical aspects of transfusion: immunology, hematology, infectious diseases, genetics, molecular biology, etc. And finally, a convivial cross-disciplinary section on training and information offers practical updates.
Readership:
"Transfusers" are many and various: anesthetists, biologists, hematologists, and blood-bank, ICU and mobile emergency specialists...