Borgna-Pignatti Caterina, Marsella Maria
Department of Medical Sciences, Section of Pediatrics, University of Ferrara, Ferrara, Italy.
Pediatrics and Adolescentology Unit, Maternal and Child Health Department, "G. Rummo" Hospital, Benevento, Italy.
Clin Ther. 2015 Dec 1;37(12):2866-77. doi: 10.1016/j.clinthera.2015.10.001. Epub 2015 Oct 27.
Iron chelation has improved survival and quality of life of patients with thalassemia major. there are currently 3 commercially available iron-chelating drugs with different pharmacokinetic and pharmacodynamic activity. The choice of adequate chelation treatment should be tailored to patient needs and based on up-to-date scientific evidence.
A review of the most recent literature was performed.
The ability of the chelators to bind the redox active component of iron, labile plasma iron, is crucial for protecting the cells. Chelation therapy should be guided by magnetic resonance imaging that permits the tailoring of therapy according to the needs of the patient because different chelators preferentially clear iron from different sites. Normal levels of body iron seem to decrease the need for hormonal and cardiac therapy.
The 3 chelators currently available have different benefits, different safety profiles, and different acceptance on the part of the patients. Good-quality, well-designed, randomized, long-term clinical trials continue to be needed.
铁螯合疗法已改善了重型地中海贫血患者的生存率和生活质量。目前有3种市售的具有不同药代动力学和药效学活性的铁螯合药物。适当的螯合治疗选择应根据患者需求并基于最新科学证据进行调整。
对最新文献进行了综述。
螯合剂结合铁的氧化还原活性成分（不稳定血浆铁）的能力对于保护细胞至关重要。螯合疗法应以磁共振成像为指导，磁共振成像能够根据患者需求调整治疗方案，因为不同的螯合剂优先从不同部位清除铁。体内铁水平正常似乎会降低对激素和心脏治疗的需求。
目前可用的3种螯合剂具有不同的益处、不同的安全性概况以及患者不同的接受度。仍然需要高质量、精心设计的随机长期临床试验。
