Transfusion and iron chelation therapy revolutionised survival and reduced morbidity in patients with transfusion-dependent beta thalassaemia major. Despite these improvements, cardiac disease remained the most common cause of death in those patients. Recently the ability to determine the degree of cardiac iron overload, through cardiac magnetic resonance imaging (CMR) has allowed more logical ...


 CADTH recommends that Ferriprox should be reimbursed by public drug plans for the treatment of patients with transfusional iron overload due to sickle cell disease (SCD) or other anemias if certain conditions are met.
 covered based on criteria used each programs deferasirox (DFX), orally administered SCD and anemias.
 only prescribed clinicians who have expertise managing cost...

Iron chelation therapy is used to reduce iron overload development due to its deposition in various organs such as liver and heart after regular transfusion. In this review, different iron chelators implicated in treatment of iron overload in various clinical conditions have been evaluated using more up-to-date studies focusing on these therapeutic agents. Deferoxamine, Deferiprone and Deferasi...

INTRODUCTION Sideroblastic cardiomyopathy secondary to repeated blood transfusions is a feared complication in thalassaemia. Control of myocardial iron is thus becoming the cornerstone of thalassaemia management. Recent evidence suggests a role for L-type Ca(2+) channels in mediating iron uptake by the heart. Blocking the cellular iron uptake through these channels may add to the benefit of the...

BACKGROUND In thalassemia major (TM), severe cardiac siderosis can be treated by continuous parenteral deferoxamine, but poor compliance, complications and deaths occur. Combined chelation therapy with deferiprone and deferoxamine is effective for moderate myocardial siderosis, but has not been prospectively examined in severe myocardial siderosis. METHODS T2* cardiovascular magnetic resonanc...

Metal toxicity represents a significant public health concern and contributes to many toxic metal-exposure related diseases. In particular, toxic metals promote the oxidative stress in stem cells and endothelial progenitor cells (EPCs). EPCs that are presented in peripheral blood function to replenish aging damaged endothelial cells and play important role in the maintenance of vasculature and ...

OBJECTIVES No consensus exists regarding the preferred treatment of childhood lead poisoning. The authors used decision analysis to compare the clinical impacts and cost-effectiveness of four management strategies for childhood lead poisoning, and to investigate how effective chelation therapy must be in reducing neuropsychologic sequelae to warrant its use. METHODS The model was based on a 2...

Masera and colleagues recently published case reports of lens opacities in three patients with b-thalassemia major that were suggested to be associated with exposure to deferasirox (ICL670, Exjade); the opacities developed 7, 16 and 26 months after initiating deferasirox therapy. These three patients are from a total of 12 being followed by the authors at their institution. This reported incide...

From 2003 to 2005, deaths of 3 individuals as a result of cardiac arrest caused by hypocalcemia during chelation therapy were reported to the Centers for Disease Control and Prevention. Two were children, both of whom were treated with edetate disodium. At the time of this writing, the adult case was still under investigation. No previous cases of death resulting from hypocalcemia during chelat...

Background Iron chelation treatment has increased life expectancy of patients with beta thalassemia major. Accurate assessement of heart iron overload is essential for managing chelation therapy. Nowadays, MRI-based iron overload quantification became the gold standard technique. However it remains too expensive in countries that cannot afford such technology, which ironically are endemic count...