Deferiprone therapy in homozygous human β-thalassemia removes erythrocyte membrane free iron and reduces KCl cotransport activity

Lucia De Franceschi, Oded Shalev, Antonio Piga, Montessar Collell, Oliviero Olivieri, Roberto Corrocher, Robert P. Hebbel, Carlo Brugnara

Research output: Contribution to journalArticlepeer-review

28 Scopus citations

Abstract

Deposition of free iron is a characteristic feature of β-thalassemia (β-thal) red blood cells believed to play an important role in the generation of oxidative injury to the cell membrane. Increased red blood cell KCl cotransport, reduced K content, and cell dehydration are also found in β-thal red blood cells. It is not known, however, whether deposition of free iron plays a role in these membrane transport changes. To explore this issue, we studied-both in vitro and in vivo-the effect on KCl cotransport of removing red blood cell membrane free iron from β-thal erythrocytes. Eleven patients with β-thal major who underwent long-term transfusion and were treated with deferiprone (75 mg/kg/day) for 9 months participated in the study. Deferiprone therapy removed membrane free iron from β-thal erythrocytes, which was followed by reduced KCl cotransport activity. The reduced KCl cotransport activity was accompanied by an increase in the red blood cell K content. These data suggest that the increased activity of KCl cotransport in β-thal red blood cells is mediated by the deposition of membrane free iron, a mechanism that may be attenuated by deferiprone therapy.

Original languageEnglish (US)
Pages (from-to)64-69
Number of pages6
JournalJournal of Laboratory and Clinical Medicine
Volume133
Issue number1
DOIs
StatePublished - 1999

Bibliographical note

Funding Information:
Supported by National Institutes of Health grants from the Heart, Lung and Blood Institute (P60-HL 15157) and the Diabetes and Digestive, and Kidney Diseases Institute (R01-DK50422); from the Ahavas Reim Foundation; and from the “Associazione Filippo Collerone”, Caltanissetta, Italy.

Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.

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