Chelation use and iron burden in North American and British thalassemia patients: A report from the thalassemia longitudinal cohort

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

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

55 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)2746-2753
Number of pages8
JournalBlood
Volume119
Issue number12
DOIs
StatePublished - Mar 22 2012

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Fingerprint Dive into the research topics of 'Chelation use and iron burden in North American and British thalassemia patients: A report from the thalassemia longitudinal cohort'. Together they form a unique fingerprint.

Cite this