Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention

Janet L. Kwiatkowski*, Alan R. Cohen, Julian Garro, Ofelia Alvarez, Ramamorrthy Nagasubramanian, Sharada Sarnaik, Alexis Thompson, Gerald M. Woods, William Schultz, Nicole Mortier, Peter Lane, Brigitta Mueller, Nancy Yovetich, Russell E. Ware

*Corresponding author for this work

Research output: Contribution to journalLetter

14 Citations (Scopus)

Abstract

Chronic transfusion reduces the risk of recurrent stroke in children with sickle cell anemia (SCA) but leads to iron loading. Management of transfusional iron overload in SCA has been reported as suboptimal, but studies characterizing monitoring and treatment practices for iron overload in children with SCA, particularly in recent years with the expansion of chelator options, are lacking. We investigated the degree of iron loading and treatment practices of 161 children with SCA receiving transfusions for a history of stroke who participated in the Stroke with Transfusions Changing to Hydroxyurea (SWiTCH) trial. Data obtained during screening, including past and entry liver iron concentration (LIC) measurements, ferritin values, and chelation were analyzed. The mean age at enrollment was 12.9 ± 4 years and the mean duration of transfusion was 7 ± 3.8 years. Baseline LIC (median 12.94 mg/g dw) and serum ferritin (median 3,164 ng/mL) were elevated. Chelation therapy was initiated after a mean of 2.6 years of transfusions. At study entry, 137 were receiving chelation, most of whom (90%) were receiving deferasirox. This study underscores the need for better monitoring of iron burden with timely treatment adjustments in chronically transfused children with SCA.

Original languageEnglish (US)
Pages (from-to)221-223
Number of pages3
JournalAmerican Journal of Hematology
Volume87
Issue number2
DOIs
StatePublished - Feb 1 2012

Fingerprint

Iron Overload
Sickle Cell Anemia
Secondary Prevention
Iron
Stroke
Ferritins
Chelation Therapy
Therapeutics
Hydroxyurea
Liver
Chelating Agents
Serum

ASJC Scopus subject areas

  • Hematology

Cite this

Kwiatkowski, J. L., Cohen, A. R., Garro, J., Alvarez, O., Nagasubramanian, R., Sarnaik, S., ... Ware, R. E. (2012). Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention. American Journal of Hematology, 87(2), 221-223. https://doi.org/10.1002/ajh.22228
Kwiatkowski, Janet L. ; Cohen, Alan R. ; Garro, Julian ; Alvarez, Ofelia ; Nagasubramanian, Ramamorrthy ; Sarnaik, Sharada ; Thompson, Alexis ; Woods, Gerald M. ; Schultz, William ; Mortier, Nicole ; Lane, Peter ; Mueller, Brigitta ; Yovetich, Nancy ; Ware, Russell E. / Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention. In: American Journal of Hematology. 2012 ; Vol. 87, No. 2. pp. 221-223.
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abstract = "Chronic transfusion reduces the risk of recurrent stroke in children with sickle cell anemia (SCA) but leads to iron loading. Management of transfusional iron overload in SCA has been reported as suboptimal, but studies characterizing monitoring and treatment practices for iron overload in children with SCA, particularly in recent years with the expansion of chelator options, are lacking. We investigated the degree of iron loading and treatment practices of 161 children with SCA receiving transfusions for a history of stroke who participated in the Stroke with Transfusions Changing to Hydroxyurea (SWiTCH) trial. Data obtained during screening, including past and entry liver iron concentration (LIC) measurements, ferritin values, and chelation were analyzed. The mean age at enrollment was 12.9 ± 4 years and the mean duration of transfusion was 7 ± 3.8 years. Baseline LIC (median 12.94 mg/g dw) and serum ferritin (median 3,164 ng/mL) were elevated. Chelation therapy was initiated after a mean of 2.6 years of transfusions. At study entry, 137 were receiving chelation, most of whom (90{\%}) were receiving deferasirox. This study underscores the need for better monitoring of iron burden with timely treatment adjustments in chronically transfused children with SCA.",
author = "Kwiatkowski, {Janet L.} and Cohen, {Alan R.} and Julian Garro and Ofelia Alvarez and Ramamorrthy Nagasubramanian and Sharada Sarnaik and Alexis Thompson and Woods, {Gerald M.} and William Schultz and Nicole Mortier and Peter Lane and Brigitta Mueller and Nancy Yovetich and Ware, {Russell E.}",
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Kwiatkowski, JL, Cohen, AR, Garro, J, Alvarez, O, Nagasubramanian, R, Sarnaik, S, Thompson, A, Woods, GM, Schultz, W, Mortier, N, Lane, P, Mueller, B, Yovetich, N & Ware, RE 2012, 'Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention', American Journal of Hematology, vol. 87, no. 2, pp. 221-223. https://doi.org/10.1002/ajh.22228

Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention. / Kwiatkowski, Janet L.; Cohen, Alan R.; Garro, Julian; Alvarez, Ofelia; Nagasubramanian, Ramamorrthy; Sarnaik, Sharada; Thompson, Alexis; Woods, Gerald M.; Schultz, William; Mortier, Nicole; Lane, Peter; Mueller, Brigitta; Yovetich, Nancy; Ware, Russell E.

In: American Journal of Hematology, Vol. 87, No. 2, 01.02.2012, p. 221-223.

Research output: Contribution to journalLetter

TY - JOUR

T1 - Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention

AU - Kwiatkowski, Janet L.

AU - Cohen, Alan R.

AU - Garro, Julian

AU - Alvarez, Ofelia

AU - Nagasubramanian, Ramamorrthy

AU - Sarnaik, Sharada

AU - Thompson, Alexis

AU - Woods, Gerald M.

AU - Schultz, William

AU - Mortier, Nicole

AU - Lane, Peter

AU - Mueller, Brigitta

AU - Yovetich, Nancy

AU - Ware, Russell E.

PY - 2012/2/1

Y1 - 2012/2/1

N2 - Chronic transfusion reduces the risk of recurrent stroke in children with sickle cell anemia (SCA) but leads to iron loading. Management of transfusional iron overload in SCA has been reported as suboptimal, but studies characterizing monitoring and treatment practices for iron overload in children with SCA, particularly in recent years with the expansion of chelator options, are lacking. We investigated the degree of iron loading and treatment practices of 161 children with SCA receiving transfusions for a history of stroke who participated in the Stroke with Transfusions Changing to Hydroxyurea (SWiTCH) trial. Data obtained during screening, including past and entry liver iron concentration (LIC) measurements, ferritin values, and chelation were analyzed. The mean age at enrollment was 12.9 ± 4 years and the mean duration of transfusion was 7 ± 3.8 years. Baseline LIC (median 12.94 mg/g dw) and serum ferritin (median 3,164 ng/mL) were elevated. Chelation therapy was initiated after a mean of 2.6 years of transfusions. At study entry, 137 were receiving chelation, most of whom (90%) were receiving deferasirox. This study underscores the need for better monitoring of iron burden with timely treatment adjustments in chronically transfused children with SCA.

AB - Chronic transfusion reduces the risk of recurrent stroke in children with sickle cell anemia (SCA) but leads to iron loading. Management of transfusional iron overload in SCA has been reported as suboptimal, but studies characterizing monitoring and treatment practices for iron overload in children with SCA, particularly in recent years with the expansion of chelator options, are lacking. We investigated the degree of iron loading and treatment practices of 161 children with SCA receiving transfusions for a history of stroke who participated in the Stroke with Transfusions Changing to Hydroxyurea (SWiTCH) trial. Data obtained during screening, including past and entry liver iron concentration (LIC) measurements, ferritin values, and chelation were analyzed. The mean age at enrollment was 12.9 ± 4 years and the mean duration of transfusion was 7 ± 3.8 years. Baseline LIC (median 12.94 mg/g dw) and serum ferritin (median 3,164 ng/mL) were elevated. Chelation therapy was initiated after a mean of 2.6 years of transfusions. At study entry, 137 were receiving chelation, most of whom (90%) were receiving deferasirox. This study underscores the need for better monitoring of iron burden with timely treatment adjustments in chronically transfused children with SCA.

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U2 - 10.1002/ajh.22228

DO - 10.1002/ajh.22228

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SP - 221

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JF - American Journal of Hematology

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