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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 (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 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 Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted February 19, 2004 Accepted April 9, 2004 Labile plasma iron (LPI) as an indicator of chelatable plasma redox activity in iron overloaded beta-thalassaemia/HbE patients treated with an oral chelator Pensri Pootrakul, William Breuer, Matias Sametband, Pornpan Sirankapracha, Chaim Hershko, and Z I Cabantchik* Institute of Science and Technology for Research and Development, Thalassemia Research Center, Salaya, Nakornpathom, Thailand Department of Biological Chemistry, Hebrew University, Jerusalem, Israel Department of Hematology, Shaare Zedek Medical Center, Jerusalem, Israel * Corresponding author; email: ioav{at}cc.huji.ac.il. Persistent levels of plasma non transferrin bound iron (NTBI) have been associated with tissue iron overload and toxicity. We characterized NTBIs susceptibility to Desferrioxamine (DCI) and redox activity (LPI) during the course of long-term, continuous L1 treatment of patients (n=17) with haemoglobin E disease and -thalassaemia (n=17). In 97% of serum samples (n=267), the LPI levels were >0.4 µM (mean ±SEM 3.1±0.2) and the % Tf saturation > 85% (111±6) whereas only in 4% of sera the LPI levels were >0.4 µM for Tf sat.<85%. Daily administration of L1 (50 mg/kg) for 13-17 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 6-8 months with a t1/2 of 2-3 months. Serum ferritin and red cell membrane associated iron followed a similar course but attained steady basal levels only after 10-12 months of continuous treatment with a t1/1 of 5-7 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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