Journal article
N. Olivieri, V. Thayalasuthan, G. Muraca, D. Weatherall, Cecilia Kim, A. Premawardhena, A. Perera, A. O’Donnell, T. S. Pierre
2010
2010
Semantic Scholar
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APA
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Olivieri, N., Thayalasuthan, V., Muraca, G., Weatherall, D., Kim, C., Premawardhena, A., … Pierre, T. S. (2010). Rates of Non-Transfusional Iron Accumulation (NTIA) In Hemoglobin E Thalassemia.
Chicago/Turabian
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Olivieri, N., V. Thayalasuthan, G. Muraca, D. Weatherall, Cecilia Kim, A. Premawardhena, A. Perera, A. O’Donnell, and T. S. Pierre. “Rates of Non-Transfusional Iron Accumulation (NTIA) In Hemoglobin E Thalassemia” (2010).
MLA
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Olivieri, N., et al. Rates of Non-Transfusional Iron Accumulation (NTIA) In Hemoglobin E Thalassemia. 2010.
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@article{n2010a,
title = {Rates of Non-Transfusional Iron Accumulation (NTIA) In Hemoglobin E Thalassemia},
year = {2010},
author = {Olivieri, N. and Thayalasuthan, V. and Muraca, G. and Weatherall, D. and Kim, Cecilia and Premawardhena, A. and Perera, A. and O’Donnell, A. and Pierre, T. S.}
}
Abstract
Abstract 5147 In patients with Hemoglobin (Hb) E thalassemia, the most common form of severe thalassemia worldwide (Weatherall DJ. Blood 2010;4331-6), early reports suggest that this disorder is associated with variable iron loading in the absence of frequent transfusions (Olivieri NF et al. Int J Ped Hem Onc 2000; 22:593-7), possibly related to the phenotypic variability of the disorder. At the National Thalassaemia Centre, Kurunegala, Sri Lanka, we have studied two groups of patients with Hb E thalassemia using spin density projection assisted R2-MRI (R2-MRI; FerriScan) (St Pierre TG et al. Blood 2005; 105:855-61). Group 1 (n=23) had received ≤ 20 transfusions (median = 9) lifelong, despite a relatively advanced age (mean ± SD, 27.5 ± 16.2 [range 7.8–57.4] years). Group 2 (n=47) had received > 20 transfusions lifelong. In Group 1, liver iron concentration (LIC) was strikingly variable (geometric mean 5.6 [range 1.0–33.0] mg Fe/g dw). LIC exceeded 7 mg Fe/g dw in 11 patients and 15 mg Fe/g dw in 4 patients. Values of serum ferritin (SF) (mean ± SD, 827 ± 611 [range 254–2484] ug/L) and serum ALT (37.6 ± 19.7 [range 12–86] U/L) did not reliably reflect the degree of elevation in LIC (R2 = 0.38, P = 0.0017 for SF; R2 = 0.12, P = 0.10 for ALT). These elevations of body iron burden were associated with evidence of organ dysfunction, with abnormal ALT (observed in 11 patients), hypothyroidism (6 patients), final adult height Rates of change of LIC in patients receiving Disclosures: St. Pierre:Resonance Health Ltd: Consultancy, Equity Ownership, Membership on an entity9s Board of Directors or advisory committees, Research Funding, Speakers Bureau; Novartis Pharmaceuticals Inc: Consultancy, Research Funding, Speakers Bureau.
