Document Detail


Chelation use and iron burden in North American and British thalassemia patients: a report from the Thalassemia Longitudinal Cohort.
MedLine Citation:
PMID:  22279056     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2012-01-25
Journal Detail:
Title:  Blood     Volume:  119     ISSN:  1528-0020     ISO Abbreviation:  Blood     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-03-23     Completed Date:  2012-05-07     Revised Date:  2013-06-26    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2746-53     Citation Subset:  AIM; IM    
Affiliation:
Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. kwiatkowski@e-mail.chop.edu
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MeSH Terms
Descriptor/Qualifier:
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:
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:
0/Iron Chelating Agents; 7439-89-6/Iron
Investigator
Investigator/Affiliation:
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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