| Chelation use and iron burden in North American and British thalassemia patients: a report from the Thalassemia Longitudinal Cohort. | |
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MedLine Citation:
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PMID: 22279056 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Morbidity and mortality in thalassemia are associated with iron burden. Recent advances in organ-specific iron imaging and the availability of oral deferasirox are expected to improve clinical care, but the extent of use of these resources and current chelation practices have not been well described. In the present study, we studied chelation use and the change in iron measurements in 327 subjects with transfusion-dependent thalassemia (mean entry age, 22.1 ± 2.5 years) from 2002-2011, with a mean follow-up of 8.0 years (range, 4.4-9.0 years). The predominant chelator currently used is deferasirox, followed by deferoxamine and then combination therapies. The use of both hepatic and cardiac magnetic resonance imaging increased more than 5-fold (P < .001) during the study period, leading to an 80% increase in the number of subjects undergoing liver iron concentration (LIC) measurements. Overall, LIC significantly improved (median, 10.7 to 5.1 mg/g dry weight, P < .001) with a nonsignificant improvement in cardiac T2* (median, 23.55 to 34.50 ms, P = .23). The percentage of patients with markers of inadequate chelation (ferritin > 2500 ng/mL, LIC > 15 mg/g dry weight, and/or cardiac T2* < 10 ms) also declined from 33% to 26%. In summary, increasing use of magnetic resonance imaging and oral chelation in thalassemia management has likely contributed to improved iron burden. |
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Authors:
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Janet L Kwiatkowski; Hae-Young Kim; Alexis A Thompson; Charles T Quinn; Brigitta U Mueller; Isaac Odame; Patricia J Giardina; Elliott P Vichinsky; Jeanne M Boudreaux; Alan R Cohen; John B Porter; Thomas Coates; Nancy F Olivieri; Ellis J Neufeld; |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2012-01-25 |
Journal Detail:
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Title: Blood Volume: 119 ISSN: 1528-0020 ISO Abbreviation: Blood Publication Date: 2012 Mar |
Date Detail:
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Created Date: 2012-03-23 Completed Date: 2012-05-07 Revised Date: 2013-05-20 |
Medline Journal Info:
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Nlm Unique ID: 7603509 Medline TA: Blood Country: United States |
Other Details:
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Languages: eng Pagination: 2746-53 Citation Subset: AIM; IM |
Affiliation:
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Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. kwiatkowski@e-mail.chop.edu |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Cohort Studies Female Humans Iron / analysis* Iron Chelating Agents / therapeutic use* Liver / chemistry*, metabolism London Longitudinal Studies Magnetic Resonance Imaging Male North America Thalassemia / drug therapy*, metabolism Young Adult |
| Grant Support | |
ID/Acronym/Agency:
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U01 HL065260/HL/NHLBI NIH HHS; U01-HL65232/HL/NHLBI NIH HHS; U01-HL65233/HL/NHLBI NIH HHS; U01-HL65238/HL/NHLBI NIH HHS; U01-HL65239/HL/NHLBI NIH HHS; U01-HL65244/HL/NHLBI NIH HHS; U01-HL72291/HL/NHLBI NIH HHS; UL1-RR-025758/RR/NCRR NIH HHS; UL1-RR024134/RR/NCRR NIH HHS; UL1-RR024996/RR/NCRR NIH HHS; ULI-RR024131/RR/NCRR NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Iron Chelating Agents; 7439-89-6/Iron |
| Investigator | |
Investigator/Affiliation:
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Ellis Neufeld / ; Jennifer Braunstein / ; Amber Smith / ; Latoya Lashley / ; Charles Quinn / ; Deborah Boger / ; Leah Adix / ; Sandra Richardson / ; Jeanne Boudreaux / ; Leann Hassen / ; Brigitta Mueller / ; Bogdan Dinu / ; Patricia Giardina / ; Dorothy Kleinert / ; Mark Weinblatt / ; Linda Skelly / ; Janet L Kwiatkowski / ; Marie Martin / ; Sage Green / ; Alexis Thompson / ; Janice Beatty / ; Diane Calamaras / ; Pauline Hess / ; Elliott Vichinsky / ; Dru Foote / ; Nancy Sweeters / ; Olivia Vega / ; Thomas Coates / ; Susan Carson / ; Tatianna Hernandez / ; Ani Menon / ; Michael Jeng / ; Kokil Bakshi / ; John Wu / ; Heather McCartney / ; Colleen Fitzgerald / ; Stephanie Badour / ; Nancy F Olivieri / ; Vivek Thayalasuthan / ; Isaac Odame / ; Manuela Merelles-Pulcini / ; John Porter / ; Cindy Bhagwandin / ; Farrukh Shah / ; Kathryn Hassell / ; Sonja McKinlay / ; Lisa Virzi / ; Felicia Trachtenberg / |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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