Practical management of iron overload

Deferiprone (GPO‐L‐ONE®) monotherapy reduces iron overload in transfusion‐dependent thalassemias: 1‐year results from a multicenter prospective, single arm, open label, dose escalating phase III pediatric study (GPO‐L‐ONE; A001) from Thailand

“…Then, it is important that iron chelation therapy should not be generalized, but rather tailored to each individual's needs based on the pre-existing iron burden, current iron overload-related complications, and continuing transfusion requirement [31]. Therefore, it is more appropriate that optimizing iron chelation therapy should primarily aim to "prevent" possible ironrelated complications by early and consistently control tissue iron levels than "rescuing" iron-related complications which health damages has already been developed [6].…”

“…Therefore patients with severe thalassemia syndrome still rely on regular blood transfusion to improve both quality of life [3] and long-term survival [4]. As a direct result, transfusional iron overload is unavoidable and this could cause fatal complications including cardiac siderosis and failure, endocrinopathies, delay puberty, liver fibrosis and failure, and increased susceptibility to infectious diseases [5][6][7]. Problems related to iron overload seem to be underestimated especially in the Asia Pacific region where there is the highest prevalence of the globin disorders in the world [8].…”

Deferasirox in MDS patients with transfusion-caused iron overload—a phase-II study

“…Ninety percent of all MDS patients with permanent anemia become dependent on blood transfusions to maintain their quality of life and to survive. With every unit of transfused blood, the patients receive approximately 200 to 250 mg iron [1]. As there is no physiological mechanism to remove an excess of body iron, regular blood transfusions inevitably lead to an accumulation of iron in various tissues in these individuals.…”

“…As there is no physiological mechanism to remove an excess of body iron, regular blood transfusions inevitably lead to an accumulation of iron in various tissues in these individuals. If untreated, iron overload may cause damages in liver, heart, pancreas, brain, joints, and endocrine tissues [2][3][4], increasing morbidity and the risk of early death [1]. As phlebotomy is not an option in MDS, the only way to remove toxic iron and to prevent complications of transfusion-dependent iron overload is, to date, rigorous iron chelation therapy [5].…”

Iron status and treatment modalities in transfusion-dependent patients with myelodysplastic syndromes

“…As one unit of blood contains between 100 and 250 mg of iron [3,18] transfusiondependent patients with MDS invariably develop iron overload at a rate of approximately 0.5mg per kg of body weight per day [19] and typically after 10-20 transfusions [2,20]. The circulating non-transferrin-bound iron produces hydroxyl and oxygen radicals that cause lipid peroxidation and damage to cell membranes, protein, and DNA [2,21], especially in target organs such as heart, liver, and endocrine glands [1,22].…”