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Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload.

Publication ,  Journal Article
Fung, EB; Harmatz, P; Milet, M; Ballas, SK; De Castro, L; Hagar, W; Owen, W; Olivieri, N; Smith-Whitley, K; Darbari, D; Wang, W; Vichinsky, E ...
Published in: Am J Hematol
April 2007

A natural history study was conducted in 142 Thalassemic (Thal), 199 transfused Sickle Cell Disease (Tx-SCD, n = 199), and 64 non-Tx-SCD subjects to describe the frequency of iron-related morbidity and mortality. Subjects recruited from 31 centers in the US, Canada or the UK were similar with respect to age (overall: 25 +/- 11 years, mean +/- SD) and gender (52% female). We found that Tx-SCD subjects were hospitalized more frequently compared with Thal or non-Tx-SCD (P < 0.001). Among those hospitalized, Tx-SCD adult subjects were more likely to be unemployed compared with Thal (RR = 1.6, 95% CI 1.0-2.5) or non-Tx-SCD (RR = 3.1, 95% CI 1.3-7.3). There was a positive relationship between the severity of iron overload, assessed by serum ferritin, and the frequency of hospitalizations (r= 0.20; P = 0.009). Twenty-three deaths were reported (6 Thal, 17 Tx-SCD) in 23.5 +/- 10 months of follow-up. Within the Tx-SCD group, those who died began transfusion (25.3 vs. 12.4 years, P < 0.001) and chelation therapy later (26.8 vs. 14.2 years, P = 0.01) compared with those who survived. The unadjusted death rate in Thal was lower (2.2/100 person years) compared with that in Tx-SCD (7.0/100 person years; RR = 0.38: 95% CI 0.12-0.99). However, no difference was observed when age at death was considered. Despite improvements in therapy, death rate in this contemporary sample of transfused adult subjects with Thal or SCD is 3 times greater than the general US population. Long term follow-up of this unique cohort of subjects will be helpful in further defining the relationship of chronic, heavy iron overload to morbidity and mortality.

Duke Scholars

Published In

Am J Hematol

DOI

ISSN

0361-8609

Publication Date

April 2007

Volume

82

Issue

4

Start / End Page

255 / 265

Location

United States

Related Subject Headings

  • United States
  • Thalassemia
  • Morbidity
  • Middle Aged
  • Male
  • Iron Overload
  • Immunology
  • Humans
  • Hospitalization
  • Ferritins
 

Citation

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Fung, E. B., Harmatz, P., Milet, M., Ballas, S. K., De Castro, L., Hagar, W., … Multi-Center Study of Iron Overload Research Group, . (2007). Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload. Am J Hematol, 82(4), 255–265. https://doi.org/10.1002/ajh.20809
Fung, Ellen B., Paul Harmatz, Meredith Milet, Samir K. Ballas, Laura De Castro, Ward Hagar, William Owen, et al. “Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload.Am J Hematol 82, no. 4 (April 2007): 255–65. https://doi.org/10.1002/ajh.20809.
Fung EB, Harmatz P, Milet M, Ballas SK, De Castro L, Hagar W, et al. Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload. Am J Hematol. 2007 Apr;82(4):255–65.
Fung, Ellen B., et al. “Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload.Am J Hematol, vol. 82, no. 4, Apr. 2007, pp. 255–65. Pubmed, doi:10.1002/ajh.20809.
Fung EB, Harmatz P, Milet M, Ballas SK, De Castro L, Hagar W, Owen W, Olivieri N, Smith-Whitley K, Darbari D, Wang W, Vichinsky E, Multi-Center Study of Iron Overload Research Group. Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload. Am J Hematol. 2007 Apr;82(4):255–265.
Journal cover image

Published In

Am J Hematol

DOI

ISSN

0361-8609

Publication Date

April 2007

Volume

82

Issue

4

Start / End Page

255 / 265

Location

United States

Related Subject Headings

  • United States
  • Thalassemia
  • Morbidity
  • Middle Aged
  • Male
  • Iron Overload
  • Immunology
  • Humans
  • Hospitalization
  • Ferritins