Transfusion and Exchange Transfusion in Sickle Cell Anaemias, with Particular Reference to Iron Metabolism

Porter, John B.; Huehns, E. R.

doi:10.1159/000205875

Cited by 47 publications

(42 citation statements)

References 23 publications

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“…19 Saturation of transferrin by excess circulating iron results in increased non transferring bound iron (NTBI), it tends to enter tissues more readily and results in formation of reactive oxygen species (ROS) such as the hydroxyl radical by Haber Weiss reaction. 20 Excess iron tends to deposit in the hepatic parenchyma causing end organ damage by ROS mediated lipid peroxidation.…”

Section: Resultsmentioning

confidence: 99%

Biochemical assessment of liver in sickle cell disease patients at a tertiary care hospital of north India

Tripathi

2016

Int J Res Med Sci

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Section: Resultsmentioning

confidence: 99%

Biochemical assessment of liver in sickle cell disease patients at a tertiary care hospital of north India

Tripathi

2016

Int J Res Med Sci

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“…Chelation guidelines for sickle-cell syndromes are similar to those for other iron overload situations as thalassemia major and multi-transfused patients [14,15]. Until recently, desferrioxamine and deferiprone were the only active iron-chelating agent approved for clinical use, mainly in the thalassemia setting [16,17].…”

Section: Discussionmentioning

confidence: 98%

Deferasirox effectively decreases iron burden in patients with double heterozygous HbS/β-thalassemia

et al. 2010

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Iron overload is present in several cases of double heterozygous HbS/beta-thalassemia (HbS/beta-thal). Deferasirox is an orally administered iron chelator which is effective on iron overloaded patients with transfusion-dependent anemia. The aim of this study was to investigate the efficacy and safety of deferasirox in HbS/beta-thal patients with iron overload. We evaluated 31 adult patients with HbS/beta-thal (14M/17F; median age 41 years) who had serum ferritin levels >1000 ng/mL and who were sporadically transfused. Total iron burden was monitored by measuring serum ferritin levels before and monthly after starting deferasirox, while liver iron concentration and cardiac iron burden were measured by magnetic resonance imaging (MRI) T2 and T2* parameters at baseline and 12 months after deferasirox treatment. Deferasirox managed to reduce the mean serum ferritin levels after 12 months of treatment from 1989±923 to 1008±776 ng/mL (P<0.001). This reduction was accompanied by a significant improvement on MRI T2* of the liver (from 3.9±3.2 to 5.8±3.1 ms; P<0.01) and by a comparable improvement of biochemical parameters of liver function. Mild nausea and diarrhoea of grade 1/2 were reported in 25% of patients within the first month of treatment, but did not re-occur. These data indicate that deferasirox provided effective control of iron levels (mainly of the liver) in minimally transfused patients with HbS/beta-thal, without significant adverse events, at similar doses to those studied widely for the treatment of patients with thalassemia syndromes.Response to Reviewers: I would like to thank the reviewer for the valuable comments about our paper entitled "Deferasirox effectively decreases iron burden in patients with double heterozygous HbS/beta-thalassemia". Please find below our answers to these comments. All changes have been bolded into the text.Reviewer #1: Comment 1. The manuscript was thoroughly revised and has remarkably improved. However, concerning two of the previous comments were incompletely answered. A more detailed answer will increase the comprehensibility of the corresponding parts of the paper. Certainly, it will be very easy for authors to add the missing information: As stated in the previous comment 5, for liver MRI, it would be helpful for the reader to know how the T2star values correspond to liver iron concentration given as mg/g or micromole/g dry or wet weight since at present the majority of institutions and colleagues are using these values and related cut-offs for the assessment of liver siderosis. Comment 2. (see previous comment 6): In "Methods" it is stated that: "The starting deferasirox dose was 10 or 20 mg/kg/day depending on baseline iron burden..." In "Results", it is written that "patients were treated with deferasirox at 10 and 20mg/kg/day? based on the number of transfusions?")? How was the "baseline iron burden defined" (according to "Results" by the number of transfusions) and what was the cut-off number of blood transfusions to decide for 20mg/kg/d? Answer: We tha...

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“…Consequently, there is no evidence of iron overload in sickle cell anemia, unless blood transfusions are given repeatedly [15,16]. About 10-20% of patients with SCD receive repeated transfusions and serum ferritin values increase proportionately with units of blood transfused [17] and liver iron concentrations [18]-provided ferritin samples are taken a sufficient interval after a vaso-occlusive crisis, when values can be greatly elevated [17]. Patients with SCD and transfusion-dependent thalassemia, if transfused at the same rate, show a similar rate of liver iron loading as well as the same risk of liver fibrosis at certain iron levels [19,20].…”

Section: Sickle Cell Diseasementioning

confidence: 99%

Concepts and goals in the management of transfusional iron overload

Porter

2007

Am. J. Hematol.

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In this review, current concepts and goals of iron chelation therapy for thalassemias, sickle cell disease, and myelodysplastic syndromes are discussed. The primary goal of iron chelation therapy is to prevent the accumulation of iron reaching harmful levels by matching iron intake from blood transfusion, with iron excreted by iron chelation. Over 30 years of experience with deferoxamine has shown iron chelation to be an effective therapeutic modality. However, chelation efficiency is limited because most of the body's iron stores are not directly chelatable, and only a small fraction of body iron is chelatable at any moment. Once iron has been deposited in organs other than the liver, for example the heart, removal by chelation is slow and inefficient. Chelation efficiency can be improved by designing regimes where chelators are available 24 hr a day to bind labile iron pools in cells and plasma. Deferoxamine has a short plasma half-life and the parenteral infusions required to achieve steady plasma levels are demanding, with consequent variable adherence to therapy. Once-daily oral administration of deferasirox achieves continuous chelation with trough concentrations sufficient to decrease plasma labile iron species progressively, and achieves an efficiency of chelation not obtainable with deferiprone or deferoxamine monotherapy. Am. J. Hematol. 82:1136Hematol. 82: -1139Hematol. 82: , 2007

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Transfusion and Exchange Transfusion in Sickle Cell Anaemias, with Particular Reference to Iron Metabolism

Cited by 47 publications

References 23 publications

Biochemical assessment of liver in sickle cell disease patients at a tertiary care hospital of north India

Biochemical assessment of liver in sickle cell disease patients at a tertiary care hospital of north India

Deferasirox effectively decreases iron burden in patients with double heterozygous HbS/β-thalassemia

Concepts and goals in the management of transfusional iron overload

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