Desferrioxamine therapy accelerates clearance of iron deposits after bone marrow transplantation for thalassaemia

Giardini, Claudio; Galimbbrti, M.; Lucarelli, G; Polchi, P; Angelucci, Emanuele; Baronciani, D; Gaziev, D; Erer, Buket; Nasa, Giorgio La; Barbanti, I; Muretto, Pietro

doi:10.1111/j.1365-2141.1995.tb08426.x

Cited by 62 publications

(26 citation statements)

References 14 publications

(3 reference statements)

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“…Deferoxamine has been proved effective and safe with many experiences. [12][13][14] In our study, we found the correlation between the number of administration and the decrease of ferritin level. Also, the patients with increased ferritin level despite of the iron-chelating therapy received less deferoxamine.…”

Section: Discussionmentioning

confidence: 68%

Effect of iron overload and iron-chelating therapy on allogeneic hematopoietic SCT in children

Lee

Kang

Kim

et al. 2009

Bone Marrow Transplant

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Section: Discussionmentioning

confidence: 68%

Effect of iron overload and iron-chelating therapy on allogeneic hematopoietic SCT in children

Lee

Kang

Kim

et al. 2009

Bone Marrow Transplant

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“…Deferoxamine, approved by FDA for the removal of iron in conditions involving iron overload, such as β-thalassemia (Giardini et al 1993), has been used primarily as a metal chelating agent to block the iron-dependent hydroxyl radical (Goldstein and Czapski 1990). It may involve an additional or alternative antioxidant mechanism which would directly scavenge free radicals, such as superoxides (O 3 ) or nitrogen dioxide ( .…”

Section: Discussionmentioning

confidence: 99%

High susceptibility of activated lymphocytes to oxidative stress-induced cell death

Degasperi

Castilho

Vercesi

2008

An. Acad. Bras. Ciênc.

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The present study provides evidence that activated spleen lymphocytes from Walker 256 tumor bearing rats are more susceptible than controls to tert-butyl hydroperoxide (t-BOOH)-induced necrotic cell death in vitro. The iron chelator and antioxidant deferoxamine, the intracellular Ca 2+ chelator BAPTA, the L-type Ca 2+ channel antagonist nifedipine or the mitochondrial permeability transition inhibitor cyclosporin A, but not the calcineurin inhibitor FK-506, render control and activated lymphocytes equally resistant to the toxic effects of t-BOOH. Incubation of activated lymphocytes in the presence of t-BOOH resulted in a cyclosporin A-sensitive decrease in mitochondrial membrane potential. These results indicate that the higher cytosolic Ca 2+ level in activated lymphocytes increases their susceptibility to oxidative stress-induced cell death in a mechanism involving the participation of mitochondrial permeability transition.

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“…The iron burden slowly decreases after HCT, but the rate of decline is accelerated by regular phlebotomy or iron chelation therapy after HCT. [49][50][51] In Pesaro, a program utilizing regular phlebotomy has been successful in Classes 2 and 3 thalassemics after HCT. Reduction in the body iron burden was accompanied by lowering of the serum alanine aminotransaminase level and improvement in the histologic changes of chronic hepatitis C, observed in 85% of these patients.…”

Section: Complications After Hct B-thalassemiamentioning

confidence: 99%

Hematopoietic cell transplantation for thalassemia and sickle cell disease: past, present and future

Bhatia

Walters²

2007

Bone Marrow Transplant

123

112

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b-Thalassemia major and sickle cell disease (SCD) are among the most common hereditary disorders worldwide. The supportive treatment of b-thalassemia major requires chronic, life-long RBC transfusions, which cause progressive iron overload and the potential for impaired endocrine, cardiac and hepatic function. The phenotype of thalassemia major is reliably predicted by its genotype. In contrast, SCD is a variable genetic disease caused by a single amino acid substitution in the b chain of human hemoglobin. Manifestations of SCD are quite varied, but generally result from the tendency of Hb S to irreversibly polymerize under physiologic stressors such as hypoxemia and acidosis. The polymerization causes perturbations in the erythrocyte integrity that promote vaso-occlusion and which manifest as clinical events such as severe painful episodes, acute chest syndrome, splenic infarction, stroke and avascular necrosis of target joints. The only cure proved for these disorders is correction of the genetic defect by allogeneic hematopoietic cell transplantation (HCT). We illustrate the pediatric experience of HCT for hemoglobinopathies and discuss how these results affect future therapeutic decisions in children who inherit these disorders. Bone Marrow Transplantation (2008) 41, 109-117; doi:10.1038/sj.bmt.1705943; published online 3 December 2007 Keywords: hematopoietic cell transplantation; hemoglobinopathies; children; sickle cell disease; thalassemia Introduction b-Thalassemia major and sickle cell disease (SCD) are hereditary anemias that decrease lifespan and reduce the quality of life. While supportive therapies are available that minimize long-term sequelae, the only cure for these disorders is allogeneic hematopoietic cell transplantation (HCT). While the outcomes after HCT are quite similar in both disorders, the decision about when and if to pursue HCT follows very different time-lines. All patients with thalassemia are at risk for transfusion-related iron overload and its attendant negative impact on clinical well-being. As a result, many clinicians strongly consider HCT as a therapeutic option in children who are less than 17 years of age and who have an HLA-identical sibling donor, due to the excellent results of HCT in this setting. However, in patients with SCD, the clinical symptoms are diverse and most clinicians delay HCT until there is evidence of severe disease, such as in those who have had a stroke, even though the results after HCT for SCD are very similar to results after HCT for thalassemia. This review presents the current state-of-the-art of HCT for b-thalassemia and SCD and discusses future directions that might improve survival and decrease morbidity. b-Thalassemia major BackgroundThalassemias result from mutations of the globin genes that cause reduced or absent hemoglobin production, reducing oxygen delivery. 1 The thalassemias are the most common single-gene disorders, with 4.83% of the world population carrying a globin gene variant. 2 To treat the anemia and restore oxygen delivery to...

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Desferrioxamine therapy accelerates clearance of iron deposits after bone marrow transplantation for thalassaemia

Cited by 62 publications

References 14 publications

Effect of iron overload and iron-chelating therapy on allogeneic hematopoietic SCT in children

Effect of iron overload and iron-chelating therapy on allogeneic hematopoietic SCT in children

High susceptibility of activated lymphocytes to oxidative stress-induced cell death

Hematopoietic cell transplantation for thalassemia and sickle cell disease: past, present and future

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