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Blood, 1 September 2004, Vol. 104, No. 5, pp. 1504-1510. Prepublished online as a Blood First Edition Paper on May 20, 2004; DOI 10.1182/blood-2004-02-0630. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-02-0630v1 104/5/1504    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Pootrakul, P. Articles by Cabantchik, Z. I. Search for Related Content PubMed PubMed Citation Articles by Pootrakul, P. Articles by Cabantchik, Z. I. Related Collections Red Cells Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  RED CELLS Labile plasma iron (LPI) as an indicator of chelatable plasma redox activity in iron-overloaded -thalassemia/HbE patients treated with an oral chelator Pensri Pootrakul, William Breuer, Matias Sametband, Pornpan Sirankapracha, Chaim Hershko, and Z. Ioav Cabantchik From the Thalassaemia Research Center, Institute of Science and Technology for Research and Development, Mahidol University, Salaya Campus, Nakornpathom, Thailand; Department of Biological Chemistry, Institute of Life Sciences, Hebrew University of Jerusalem, Israel; and Department of Hematology, Shaare Zedek Medical Center, Jerusalem, Israel. Persistent levels of plasma nontransferrin bound iron (NTBI) have been associated with tissue iron overload and toxicity. We characterized NTBI's susceptibility to deferoxamine (directly chelatable iron [DCI]) and redox activity (labile plasma iron [LPI]) during the course of long-term, continuous L1 (deferiprone) treatment of patients with hemoglobin E disease and -thalassemia (n = 17). In 97% of serum samples (n = 267), the LPI levels were more than 0.4 µM (mean ± SEM, 3.1 ± 0.2 µM) and the percent transferrin (Tf) saturation more than 85 (111 ± 6), whereas only in 4% of sera were the LPI levels more than 0.4 µM for Tf saturation less than 85%. Daily administration of L1 (50 mg/kg) for 13 to 17 months caused both LPI and DCI to decrease from respective initial 5.1 ± 0.5 and 5.4 ± 0.6 µM to steady mean levels of 2.18 ± 0.24 and 2.81 ± 0.14 µM. The steady lowest levels of LPI and DCI were attained after 6 to 8 months, with a half time (t1/2) of 2 to 3 months. Serum ferritin and red cell membrane–associated iron followed a similar course but attained steady basal levels only after 10 to 12 months of continuous treatment, with a t1/2 of 5 to 7 months. These studies indicate that LPI and DCI can serve as early indicators of iron overload and as measures for the effectiveness of iron chelation in reducing potentially toxic iron in the plasma. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: P. B. Walter, E. A. Macklin, J. Porter, P. Evans, J. L. Kwiatkowski, E. J. Neufeld, T. Coates, P. J. Giardina, E. Vichinsky, N. Olivieri, et al. Inflammation and oxidant-stress in {beta}-thalassemia patients treated with iron chelators deferasirox (ICL670) or deferoxamine: an ancillary study of the Novartis CICL670A0107 trial Haematologica, June 1, 2008; 93(6): 817 - 825. [Abstract] [Full Text] [PDF] S. Gardenghi, M. F. Marongiu, P. Ramos, E. Guy, L. Breda, A. Chadburn, Y. Liu, N. Amariglio, G. Rechavi, E. A. Rachmilewitz, et al. Ineffective erythropoiesis in -thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation of ferroportin Blood, June 1, 2007; 109(11): 5027 - 5035. [Abstract] [Full Text] [PDF] C. Le Lan, O. Loreal, T. Cohen, M. Ropert, H. Glickstein, F. Laine, M. Pouchard, Y. Deugnier, A. Le Treut, W. Breuer, et al. Redox active plasma iron in C282Y/C282Y hemochromatosis Blood, June 1, 2005; 105(11): 4527 - 4531. [Abstract] [Full Text] [PDF] A. R. Cohen, R. Galanello, D. J. Pennell, M. J. Cunningham, and E. Vichinsky Thalassemia Hematology, January 1, 2004; 2004(1): 14 - 34. [Abstract] [Full Text] [PDF]

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