... in iron overload, which is associated with an increased oxidative stress state. Iron-chelation therapy is applied to attenuate the toxic effects of this state. Deferiprone (DFP) is an oral iron chelator, which is not commonly used in this patient population, due to safety concerns, mainly agranulocytosis...

... overload on the mobilization of peripheral blood stem cells (PBSCs) in pediatric patients with β-thalassemia major (TM). Methods: We retrospectively reviewed the records of 226 patients with TM from whom PBSCs were collected. Iron overload was based on serum ferritin level, and liver and cardiac iron...

... insight for improving the clinical management of patients with different types of thalassemia. Hemoglobin H disease β-Thalassemia Splenectomy Iron overload Thalassemia is one of the most common monogenic genetic disorders worldwide, with a region-specific distribution that generally overlaps...

... months in grade 2; 33.2 ± 16.4%, median 144 ± 18 months in grade 1; p = 0.011); bone marrow iron overload ( p = 0.003; HR 1.7) and transfusion dependency (0.001; HR 2.6) negatively impacted on survival. We suggest that a higher grade of iron storage at diagnosis can impact on the outcome in MDS patients...

...Soumitra Tole; Ali Amid; Jillian Baker; Kevin Kuo; Jakob Pugi; Manuel Carcao Hereditary spherocytosis (HS) is a common inherited haemolytic anaemia and has great variability in its presentation. Non-transfusion iron overload in HS has only been reported with co-inheritance of hereditary...

...Yali Du; Zhangbiao Long; Miao Chen; Bing Han; Bo Hou; Feng Feng Background: This study focuses on the iron overload (IOL) of patients with transfused aplastic anemia (AA) or a low/intermediate-1 risk of myelodysplastic syndrome (MDS). Methods: Ninety-two AA or MDS patients with IOL were...

...Claire Sheeran; Donald K. Bowden; Sant-Rayn Pasricha; Ken Cheng; Giovanni Romanelli; Roger E. Peverill Background/Aims: Hemoglobin H (HbH) disease is associated with iron overload, but whether this results in serious cardiac or vascular sequelae is unresolved. Methods: We identified 39 adult...

... for iron overload, (2) it could confirm the association between free iron and early HSCT toxicity and complications and (3) it is a potential target for iron chelators. The purpose of this study was to evaluate the kinetics of LPI levels in HSCT patients by obtaining sequential LPI determinations...

... and safety of oral iron chelators, and noninvasive techniques for the assessment of iron overload are translated into better patient outcomes. The construction of TM management guidelines facilitated the incorporation of such evidence into practice. However, as several aspects of the management of TM remain...

...Rodolfo Cancado; Maria Cristina A. Olivato; Paula Bruniera; Gilberto Szarf; Roberto de Moraes Bastos; Murilo Rezende Melo; Carlos Chiattone The efficacy and safety of a 2-year treatment with deferasirox was evaluated in 31 patients with sickle cell anemia and transfusional iron overload. At 24...

... (exon 7, c.809A→T), a mutation previously reported only in 1 black patient with iron overload who resided in the Republic of South Africa. The present patient was also heterozygous for: heme transporter FLVCR1 novel allele P542S (exon 10, 1624C→T); FLVCR1 T544M (rs3207090); hemopexin (HPX) R371W...

... of deferasirox and its iron complex in rats. Drug Metab Dispos 2008;36:2523–2538. Deferasirox Iron overload Pregnancy The therapeutic benefit of tailored iron chelation therapy and regular transfusions in patients with thalassemia major has significantly improved life expectancy; the number...

...A. Quarta; A. Melpignano; G. Quarta Iron overload sometimes complicates the clinical course of bone marrow transplantation and can cause damage to liver and heart function. A patient with post-transplantation secondary hemochromatosis was treated with deferasirox, which not only normalized ferritin...

...-thalassaemia, perhaps due to the toxic effect of iron overload on adipose tissue. Paradoxically, adiponectin levels are high and positively correlated with endothelin-1, raising questions about the pro- or anti-inflammatory role of this adipocytokine in beta-thalassaemia. 14 04 2010 3 08 2010 2...

...Ali Taher; Abdullah Al Jefri; Mohsen Saleh Elalfy; Kusai Al Zir; Shahina Daar; Diana Rofail; Jean François Baladi; Dany Habr; Ulrike Kriemler-Krahn; Amal El-Beshlawy Patient-reported outcomes of once-daily oral deferasirox (Exjade®) in iron-overloaded patients with β-thalassemia not achieving...

... therapy although a randomized trial of phlebotomy versus no treatment has never been reported. Since the discovery of the HFE gene in 1996, it has been possible to predict the risk of developing iron overload by a simple blood test to detect C282Y homozygotes of the HFE gene. The application...

...M. Westerhausen; G. Meuret A 71-year-old woman showed a highly unusual pattern of iron distribution in the organism which was associated with iron overload. The hallmark of this disease was an extreme hypersiderinemia, the serum iron reaching about 800 μ g/l00 ml. There was a pigment cirrhosis...

... levels and unit of blood transfused on the other. The results of this study suggest that iron overload could be a cause of damage in the high frequency elements of the auditory mechanism. Intermittent hypoxia and slow 8th nerve compression due to bony hypertrophy as causes of auditory involvement...

... was significantly higher than normal values indicating tissue excess and came down sharply after treatment with iron. In thalassaemia, associated presumably with iron overload, ascorbic acid status of platelets was definitely lower than normal indicating tissue deficiency. Leucocytic level of ascorbic acid...

... active hepatitis, 23 with chronic persistent hepatitis, 22 with siderosis). Viral marker studies showed only 3 cases with both HBsAg and anti-HBc posiüvity in the serum, while the others had anti-HBc and anti-HBs or only anti-HBs or no B viral markers. A significantly higher iron overload was found...