Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury

Thomas V. Adamkiewicz, Miguel R. Abboud, Carole Paley, Nancy Olivieri, Melanie Kirby-Allen, Elliott Vichinsky, James F. Casella, Ofelia A. Alvarez, Julio C. Barredo, Margaret T. Lee, Rathi V. Iyer, Abdullah Kutlar, Kathleen M. McKie, Virgil McKie, Nadine Odo, Beatrice Gee, Janet L. Kwiatkowski, Gerald M. Woods, Thomas Coates, Winfred Wang & 1 others Robert J. Adams

Research output: Contribution to journalArticle

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Abstract

Chronic blood transfusion is increasingly indicated in patients with sickle cell disease. Measuring resulting iron overload remains a challenge. Children without viral hepatitis enrolled in 2 trials for stroke prevention were examined for iron overload (STOP and STOP2; n = 271). Most received desferrioxamine chelation. Serum ferritin (SF) changes appeared nonlinear compared with prechelation estimated transfusion iron load (TIL) or with liver iron concentrations (LICs). Averaged correlation coefficient between SF and TIL (patients/observations, 26 of 164) was r = 0.70; between SF and LIC (patients/observations, 33 of 47) was r = 0.55. In mixed models, SF was associated with LIC (P = .006), alanine transaminase (P = .025), and weight (P = .026). Most patients with SF between 750 and 1500 ng/mL had a TIL between 25 and 100 mg/kg (72.8% ± 5.9%; patients/observations, 24 of 50) or an LIC between 2.5 and 10 mg/g dry liver weight (75% ± 0%; patients/ observations, 8 of 9). Most patients with SF of 3000 ng/mL or greater had a TIL of 100 mg/kg or greater (95.3% ± 6.7%; patients/observations, 7 of 16) or an LIC of 10 mg/g dry liver weight or greater (87.7% ± 4.3%; patients/observations, 11 of 18). Although SF changes are nonlinear, levels less than 1500 ng/mL indicated mostly acceptable iron overload; levels of 3000 ng/mL or greater were specific for significant iron overload and were associated with liver injury. However, to determine accurately iron overload in patients with intermediately elevated SF levels, other methods are required. These trials are registered at www.clinicaltrials.gov as #NCT00000592 and #NCT00006182.

Original languageEnglish (US)
Pages (from-to)4632-4638
Number of pages7
JournalBlood
Volume114
Issue number21
DOIs
StatePublished - Nov 19 2009

Fingerprint

Sickle Cell Anemia
Ferritins
Blood Transfusion
Liver
Blood
Iron
Iron Overload
Wounds and Injuries
Serum
Weights and Measures
Deferoxamine
Alanine Transaminase
Hepatitis
Stroke
Chelation

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Adamkiewicz, T. V., Abboud, M. R., Paley, C., Olivieri, N., Kirby-Allen, M., Vichinsky, E., ... Adams, R. J. (2009). Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. Blood, 114(21), 4632-4638. https://doi.org/10.1182/blood-2009-02-203323

Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. / Adamkiewicz, Thomas V.; Abboud, Miguel R.; Paley, Carole; Olivieri, Nancy; Kirby-Allen, Melanie; Vichinsky, Elliott; Casella, James F.; Alvarez, Ofelia A.; Barredo, Julio C.; Lee, Margaret T.; Iyer, Rathi V.; Kutlar, Abdullah; McKie, Kathleen M.; McKie, Virgil; Odo, Nadine; Gee, Beatrice; Kwiatkowski, Janet L.; Woods, Gerald M.; Coates, Thomas; Wang, Winfred; Adams, Robert J.

In: Blood, Vol. 114, No. 21, 19.11.2009, p. 4632-4638.

Research output: Contribution to journalArticle

Adamkiewicz, TV, Abboud, MR, Paley, C, Olivieri, N, Kirby-Allen, M, Vichinsky, E, Casella, JF, Alvarez, OA, Barredo, JC, Lee, MT, Iyer, RV, Kutlar, A, McKie, KM, McKie, V, Odo, N, Gee, B, Kwiatkowski, JL, Woods, GM, Coates, T, Wang, W & Adams, RJ 2009, 'Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury', Blood, vol. 114, no. 21, pp. 4632-4638. https://doi.org/10.1182/blood-2009-02-203323
Adamkiewicz, Thomas V. ; Abboud, Miguel R. ; Paley, Carole ; Olivieri, Nancy ; Kirby-Allen, Melanie ; Vichinsky, Elliott ; Casella, James F. ; Alvarez, Ofelia A. ; Barredo, Julio C. ; Lee, Margaret T. ; Iyer, Rathi V. ; Kutlar, Abdullah ; McKie, Kathleen M. ; McKie, Virgil ; Odo, Nadine ; Gee, Beatrice ; Kwiatkowski, Janet L. ; Woods, Gerald M. ; Coates, Thomas ; Wang, Winfred ; Adams, Robert J. / Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. In: Blood. 2009 ; Vol. 114, No. 21. pp. 4632-4638.
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AU - Olivieri, Nancy

AU - Kirby-Allen, Melanie

AU - Vichinsky, Elliott

AU - Casella, James F.

AU - Alvarez, Ofelia A.

AU - Barredo, Julio C.

AU - Lee, Margaret T.

AU - Iyer, Rathi V.

AU - Kutlar, Abdullah

AU - McKie, Kathleen M.

AU - McKie, Virgil

AU - Odo, Nadine

AU - Gee, Beatrice

AU - Kwiatkowski, Janet L.

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N2 - Chronic blood transfusion is increasingly indicated in patients with sickle cell disease. Measuring resulting iron overload remains a challenge. Children without viral hepatitis enrolled in 2 trials for stroke prevention were examined for iron overload (STOP and STOP2; n = 271). Most received desferrioxamine chelation. Serum ferritin (SF) changes appeared nonlinear compared with prechelation estimated transfusion iron load (TIL) or with liver iron concentrations (LICs). Averaged correlation coefficient between SF and TIL (patients/observations, 26 of 164) was r = 0.70; between SF and LIC (patients/observations, 33 of 47) was r = 0.55. In mixed models, SF was associated with LIC (P = .006), alanine transaminase (P = .025), and weight (P = .026). Most patients with SF between 750 and 1500 ng/mL had a TIL between 25 and 100 mg/kg (72.8% ± 5.9%; patients/observations, 24 of 50) or an LIC between 2.5 and 10 mg/g dry liver weight (75% ± 0%; patients/ observations, 8 of 9). Most patients with SF of 3000 ng/mL or greater had a TIL of 100 mg/kg or greater (95.3% ± 6.7%; patients/observations, 7 of 16) or an LIC of 10 mg/g dry liver weight or greater (87.7% ± 4.3%; patients/observations, 11 of 18). Although SF changes are nonlinear, levels less than 1500 ng/mL indicated mostly acceptable iron overload; levels of 3000 ng/mL or greater were specific for significant iron overload and were associated with liver injury. However, to determine accurately iron overload in patients with intermediately elevated SF levels, other methods are required. These trials are registered at www.clinicaltrials.gov as #NCT00000592 and #NCT00006182.

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