Yazarlar : Kwiatkowski JL, Kim HY, Thompson AA, Quinn CT, Mueller BU, Odame I, Giardina PJ, Vichinsky EP, Boudreaux JM, Cohen AR, Porter JB, Coates T, Olivieri NF, Neufeld EJ.
Yayın : Blood.
Pubmed Linki : http://www.ncbi.nlm.nih.gov/pubmed/22279056
Konu : Talasemi
Literatür İçeriği :
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 utilization of these resources and current chelation practices have not been well described. 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 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 MRI increased more than five-fold (p<0.001) during the study period leading to an 80% rise 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 (dw), p<0.001] with a non-significant improvement in cardiac T2* (median 23.55 to 34.50 ms, p= 0.23). The percentage of patients with markers of inadequate chelation (ferritin >2500 ng/mL, LIC >15 mg/g dw and/or cardiac T2* <10 ms) also declined from 33 to 26%. In summary, increasing use of MRI and oral chelation in thalassemia management likely has contributed to improved iron burden.
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