Deferiprone versus desferrioxamine in thalassaemia, and T2* validation and utility

Brittenham, GM; Nathan, DG; Olivieri, NF; Pippard, M. J.; Weatherall, D. J.

doi:10.1016/s0140-6736(03)12223-4

Cited by 28 publications

(20 citation statements)

References 5 publications

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“…Iron was mobilized more readily from the liver than the heart as established by MRI T2 Ã measurements. Some evidence suggests that mobilization of iron from the myocardium is more efficient with the low-molecular-weight chelator deferiprone (Brittenham et al 2003), and that desferrioxamine and deferiprone administration together are more effective than desferrioxamine alone in reversing cardiac dysfunction as shown in a randomized trial (Tanner et al 2007). Deferasirox has also been shown to reduce and prevent cardiac iron overload (Pennell et al 2010(Pennell et al , 2011.…”

Section: Cardiac Manifestationsmentioning

confidence: 99%

Pathophysiology and Clinical Manifestations of the -Thalassemias

Nienhuis

Nathan

2012

Cold Spring Harbor Perspectives in Medicine

158

111

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The b-thalassemia syndromes reflect deficient or absent b-globin synthesis usually owing to a mutation in the b-globin locus. The relative excess of a-globin results in the formation of insoluble aggregates leading to ineffective erythropoiesis and shortened red cell survival. A relatively high capacity for fetal hemoglobin synthesis is a major genetic modifier of disease severity, with polymorphisms in other genes also having a significant role. Iron overload secondary to enhanced absorption and red cell transfusions causes an increase in liver iron and in various other tissues, leading to endocrine and cardiac dysfunction. Modern chelation regimens are effective in removing iron and preserving or restoring organ function.

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Section: Cardiac Manifestationsmentioning

confidence: 99%

Pathophysiology and Clinical Manifestations of the -Thalassemias

Nienhuis

Nathan

2012

Cold Spring Harbor Perspectives in Medicine

158

111

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“…Cardiac T2* was initially received with considerable skepticism [56][57][58]. Since cardiac biopsy is more variable and more dangerous than hepatic biopsy, direct tissue validation of cardiac T2* was a challenge [59,60].…”

Section: Validation Of Cardiac Iron Measurementsmentioning

confidence: 99%

Magnetic resonance imaging measurement of iron overload

Wood

2007

Current Opinion in Hematology

215

197

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Purpose of review-To highlight recent advances in magnetic resonance imaging estimation of somatic iron overload. This review will discuss the need and principles of magnetic resonance imaging-based iron measurements, the validation of liver and cardiac iron measurements, and the key institutional requirements for implementation.Recent findings-Magnetic resonance imaging assessment of liver and cardiac iron has achieved critical levels of availability, utility, and validity to serve as the primary endpoint of clinical trials. Calibration curves for the magnetic resonance imaging parameters R2 and R2* (or their reciprocals, T2 and T2*) have been developed for the liver and the heart. Interscanner variability for these techniques has proven to be on the order of 5-7%.Summary-Magnetic resonance imaging assessment of tissue iron is becoming increasingly important in the management of transfusional iron load because it is noninvasive, relatively widely available and offers a window into presymptomatic organ dysfunction. The techniques are highly reproducible within and across machines and have been chemically validated in the liver and the heart. These techniques will become the standard of care as industry begins to support the acquisition and postprocessing software.

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“…Low T2* values (Ͻ 8 ms compared with normal [Ͼ 20 ms]) are related to risk of heart failure and death in iron-overloaded thalassemia patients. 20 T2* reports initially raised concerns about whether it is a good surrogate for cardiac iron concentration 25 for technical reasons as well as the paradox that liver iron correlated poorly with cardiac T2*. These points have largely been settled.…”

Section: Clinical Assessments Of Chelator Efficacy and Novel Noninvasmentioning

confidence: 99%

“…This prospective trial validates the prior retrospective series of Anderson et al 29 that had been controversial. 25 A potential weakness of the trial is the use of the surrogate T2* as primary outcome, because the clinical significance of a few more milliseconds within the mildly abnormal range is unknown if taken alone. However, taken together with in vitro data and with the epidemiologic data of Borgna-Pignatti et al, 2 this trial provides For personal use only.…”

Section: Randomized Clinical Trial Of Deferiprone Versus Deferoxaminementioning

confidence: 99%