Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions

Ellen B. Fung, Paul R. Harmatz, Meredith Milet, Vinod Balasa, Samir K. Ballas, James F Casella, Lee Hilliard, Abdullah Kutlar, Kenneth L. McClain, Nancy F. Olivieri, John B. Porter, Elliott P. Vichinsky, Rita Bellevue, Thomas Coates, Deepika Darbari, Carlton Davis, Laura DeCastro, Patricia Giardina, Jeffrey Hord, Michael JengMelanie Kirby, Robert Mignaca, William Mentzer, Nancy Olivieri, William Owen, Charles Pegelow, John Porter, Gloria Ramirez, Mark Ranalli, Spreedhar Rao, Charles Scher, Frank Shafer, Mary Gail Smith, Kim Smith-Whitney, Alexis Thompson, Winfred Wang

Research output: Contribution to journalArticle

Abstract

BACKGROUND: Transfusion therapy is frequently used to prevent morbidity in sickle cell disease (SCD), and subsequent iron overload is common. The objective of this study was to evaluate the current standard of care in monitoring iron overload and related complications in patients with SCD compared to thalassemia (Thal). STUDY DESIGN AND METHODS: A cross-sectional study was conducted at 31 hematology clinics in the United States, Canada, or the United Kingdom. Patients who received transfusions with a mean serum ferritin level of least 2000 ng per mL were eligible. A total of 199 patients with SCD (113 female; 24.9 ± 13.2 years) and 142 with Thal (66 female; 25.8 ± 8.1 years) were recruited, and data were collected between 2001 and 2003 by interview and medical record review. RESULTS: Although both groups were recruited on the basis of significant iron overload, the likelihood of performing a liver biopsy for routine iron monitoring was significantly higher (odds ratio [OR], 3.4; 95% confidence interval [CI], 2.2-5.3) in Thal than SCD. Thal patients were also more likely to be screened for iron-related organ injury including an echocardiograph for cardiomyopathy (OR, 2.6; p <0.001; 95% CI, 1.6-4.2), alanine aminotransferase for liver function (OR, 8.3; CI, 1.05-64.4), and thyroid-stimulating hormone for hypothyroidism (OR, 12.3; CI, 7.0-21.5). For adult SCD patients, those maintained on simple transfusion with a serum ferritin level of greater than 2500 ng per mL were the least likely to have a liver biopsy (p <0.03). CONCLUSIONS: These data highlight the unsystematic monitoring of iron and related organ injury in SCD. Until the relationship between iron and related comorbidities is better understood, routine monitoring of iron overload in SCD patients who receive transfusions should be considered a standard part of clinical care.

Original languageEnglish (US)
Pages (from-to)1971-1980
Number of pages10
JournalTransfusion
Volume48
Issue number9
DOIs
StatePublished - Sep 2008

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Iron Overload
Thalassemia
Sickle Cell Anemia
Iron
Odds Ratio
Confidence Intervals
Ferritins
Liver
Biopsy
Wounds and Injuries
Hematology
Thyrotropin
Standard of Care
Hypothyroidism
Serum
Alanine Transaminase
Cardiomyopathies
Canada
Medical Records
Comorbidity

ASJC Scopus subject areas

  • Hematology
  • Immunology

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Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions. / Fung, Ellen B.; Harmatz, Paul R.; Milet, Meredith; Balasa, Vinod; Ballas, Samir K.; Casella, James F; Hilliard, Lee; Kutlar, Abdullah; McClain, Kenneth L.; Olivieri, Nancy F.; Porter, John B.; Vichinsky, Elliott P.; Bellevue, Rita; Coates, Thomas; Darbari, Deepika; Davis, Carlton; DeCastro, Laura; Giardina, Patricia; Hord, Jeffrey; Jeng, Michael; Kirby, Melanie; Mignaca, Robert; Mentzer, William; Olivieri, Nancy; Owen, William; Pegelow, Charles; Porter, John; Ramirez, Gloria; Ranalli, Mark; Rao, Spreedhar; Scher, Charles; Shafer, Frank; Smith, Mary Gail; Smith-Whitney, Kim; Thompson, Alexis; Wang, Winfred.

In: Transfusion, Vol. 48, No. 9, 09.2008, p. 1971-1980.

Research output: Contribution to journalArticle

Fung, EB, Harmatz, PR, Milet, M, Balasa, V, Ballas, SK, Casella, JF, Hilliard, L, Kutlar, A, McClain, KL, Olivieri, NF, Porter, JB, Vichinsky, EP, Bellevue, R, Coates, T, Darbari, D, Davis, C, DeCastro, L, Giardina, P, Hord, J, Jeng, M, Kirby, M, Mignaca, R, Mentzer, W, Olivieri, N, Owen, W, Pegelow, C, Porter, J, Ramirez, G, Ranalli, M, Rao, S, Scher, C, Shafer, F, Smith, MG, Smith-Whitney, K, Thompson, A & Wang, W 2008, 'Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions', Transfusion, vol. 48, no. 9, pp. 1971-1980. https://doi.org/10.1111/j.1537-2995.2008.01775.x
Fung, Ellen B. ; Harmatz, Paul R. ; Milet, Meredith ; Balasa, Vinod ; Ballas, Samir K. ; Casella, James F ; Hilliard, Lee ; Kutlar, Abdullah ; McClain, Kenneth L. ; Olivieri, Nancy F. ; Porter, John B. ; Vichinsky, Elliott P. ; Bellevue, Rita ; Coates, Thomas ; Darbari, Deepika ; Davis, Carlton ; DeCastro, Laura ; Giardina, Patricia ; Hord, Jeffrey ; Jeng, Michael ; Kirby, Melanie ; Mignaca, Robert ; Mentzer, William ; Olivieri, Nancy ; Owen, William ; Pegelow, Charles ; Porter, John ; Ramirez, Gloria ; Ranalli, Mark ; Rao, Spreedhar ; Scher, Charles ; Shafer, Frank ; Smith, Mary Gail ; Smith-Whitney, Kim ; Thompson, Alexis ; Wang, Winfred. / Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions. In: Transfusion. 2008 ; Vol. 48, No. 9. pp. 1971-1980.
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abstract = "BACKGROUND: Transfusion therapy is frequently used to prevent morbidity in sickle cell disease (SCD), and subsequent iron overload is common. The objective of this study was to evaluate the current standard of care in monitoring iron overload and related complications in patients with SCD compared to thalassemia (Thal). STUDY DESIGN AND METHODS: A cross-sectional study was conducted at 31 hematology clinics in the United States, Canada, or the United Kingdom. Patients who received transfusions with a mean serum ferritin level of least 2000 ng per mL were eligible. A total of 199 patients with SCD (113 female; 24.9 ± 13.2 years) and 142 with Thal (66 female; 25.8 ± 8.1 years) were recruited, and data were collected between 2001 and 2003 by interview and medical record review. RESULTS: Although both groups were recruited on the basis of significant iron overload, the likelihood of performing a liver biopsy for routine iron monitoring was significantly higher (odds ratio [OR], 3.4; 95{\%} confidence interval [CI], 2.2-5.3) in Thal than SCD. Thal patients were also more likely to be screened for iron-related organ injury including an echocardiograph for cardiomyopathy (OR, 2.6; p <0.001; 95{\%} CI, 1.6-4.2), alanine aminotransferase for liver function (OR, 8.3; CI, 1.05-64.4), and thyroid-stimulating hormone for hypothyroidism (OR, 12.3; CI, 7.0-21.5). For adult SCD patients, those maintained on simple transfusion with a serum ferritin level of greater than 2500 ng per mL were the least likely to have a liver biopsy (p <0.03). CONCLUSIONS: These data highlight the unsystematic monitoring of iron and related organ injury in SCD. Until the relationship between iron and related comorbidities is better understood, routine monitoring of iron overload in SCD patients who receive transfusions should be considered a standard part of clinical care.",
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T1 - Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions

AU - Fung, Ellen B.

AU - Harmatz, Paul R.

AU - Milet, Meredith

AU - Balasa, Vinod

AU - Ballas, Samir K.

AU - Casella, James F

AU - Hilliard, Lee

AU - Kutlar, Abdullah

AU - McClain, Kenneth L.

AU - Olivieri, Nancy F.

AU - Porter, John B.

AU - Vichinsky, Elliott P.

AU - Bellevue, Rita

AU - Coates, Thomas

AU - Darbari, Deepika

AU - Davis, Carlton

AU - DeCastro, Laura

AU - Giardina, Patricia

AU - Hord, Jeffrey

AU - Jeng, Michael

AU - Kirby, Melanie

AU - Mignaca, Robert

AU - Mentzer, William

AU - Olivieri, Nancy

AU - Owen, William

AU - Pegelow, Charles

AU - Porter, John

AU - Ramirez, Gloria

AU - Ranalli, Mark

AU - Rao, Spreedhar

AU - Scher, Charles

AU - Shafer, Frank

AU - Smith, Mary Gail

AU - Smith-Whitney, Kim

AU - Thompson, Alexis

AU - Wang, Winfred

PY - 2008/9

Y1 - 2008/9

N2 - BACKGROUND: Transfusion therapy is frequently used to prevent morbidity in sickle cell disease (SCD), and subsequent iron overload is common. The objective of this study was to evaluate the current standard of care in monitoring iron overload and related complications in patients with SCD compared to thalassemia (Thal). STUDY DESIGN AND METHODS: A cross-sectional study was conducted at 31 hematology clinics in the United States, Canada, or the United Kingdom. Patients who received transfusions with a mean serum ferritin level of least 2000 ng per mL were eligible. A total of 199 patients with SCD (113 female; 24.9 ± 13.2 years) and 142 with Thal (66 female; 25.8 ± 8.1 years) were recruited, and data were collected between 2001 and 2003 by interview and medical record review. RESULTS: Although both groups were recruited on the basis of significant iron overload, the likelihood of performing a liver biopsy for routine iron monitoring was significantly higher (odds ratio [OR], 3.4; 95% confidence interval [CI], 2.2-5.3) in Thal than SCD. Thal patients were also more likely to be screened for iron-related organ injury including an echocardiograph for cardiomyopathy (OR, 2.6; p <0.001; 95% CI, 1.6-4.2), alanine aminotransferase for liver function (OR, 8.3; CI, 1.05-64.4), and thyroid-stimulating hormone for hypothyroidism (OR, 12.3; CI, 7.0-21.5). For adult SCD patients, those maintained on simple transfusion with a serum ferritin level of greater than 2500 ng per mL were the least likely to have a liver biopsy (p <0.03). CONCLUSIONS: These data highlight the unsystematic monitoring of iron and related organ injury in SCD. Until the relationship between iron and related comorbidities is better understood, routine monitoring of iron overload in SCD patients who receive transfusions should be considered a standard part of clinical care.

AB - BACKGROUND: Transfusion therapy is frequently used to prevent morbidity in sickle cell disease (SCD), and subsequent iron overload is common. The objective of this study was to evaluate the current standard of care in monitoring iron overload and related complications in patients with SCD compared to thalassemia (Thal). STUDY DESIGN AND METHODS: A cross-sectional study was conducted at 31 hematology clinics in the United States, Canada, or the United Kingdom. Patients who received transfusions with a mean serum ferritin level of least 2000 ng per mL were eligible. A total of 199 patients with SCD (113 female; 24.9 ± 13.2 years) and 142 with Thal (66 female; 25.8 ± 8.1 years) were recruited, and data were collected between 2001 and 2003 by interview and medical record review. RESULTS: Although both groups were recruited on the basis of significant iron overload, the likelihood of performing a liver biopsy for routine iron monitoring was significantly higher (odds ratio [OR], 3.4; 95% confidence interval [CI], 2.2-5.3) in Thal than SCD. Thal patients were also more likely to be screened for iron-related organ injury including an echocardiograph for cardiomyopathy (OR, 2.6; p <0.001; 95% CI, 1.6-4.2), alanine aminotransferase for liver function (OR, 8.3; CI, 1.05-64.4), and thyroid-stimulating hormone for hypothyroidism (OR, 12.3; CI, 7.0-21.5). For adult SCD patients, those maintained on simple transfusion with a serum ferritin level of greater than 2500 ng per mL were the least likely to have a liver biopsy (p <0.03). CONCLUSIONS: These data highlight the unsystematic monitoring of iron and related organ injury in SCD. Until the relationship between iron and related comorbidities is better understood, routine monitoring of iron overload in SCD patients who receive transfusions should be considered a standard part of clinical care.

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