Literature DB >> 17457853

Liver biopsy results in patients with sickle cell disease on chronic transfusions: poor correlation with ferritin levels.

Lina B Karam1, Deborah Disco, Sherron M Jackson, David Lewin, Virgil McKie, Robert D Baker, Susan S Baker, Joseph H Laver, Paul J Nietert, Miguel R Abboud.   

Abstract

BACKGROUND: Chronic transfusions are effective in preventing stroke and other complications of sickle cell disease. The aim of this study was to determine whether serum ferritin levels correlated with liver iron content in sickle cell patients on chronic transfusion. PROCEDURE: Forty-four liver biopsy specimens from 38 patients with homozygous sickle cell anemia (HbSS) and one patient with sickle thalassemia receiving chronic transfusions were studied. Five patients underwent a second liver biopsy for follow up. Three ferritin measurements were used to calculate a mean for each patient. The association between serum ferritin levels and liver iron quantitation was measured using the Spearman rank correlation, and sensitivity and specificity were determined for selected threshold values of serum ferritin.
RESULTS: Serum ferritin levels ranged from 515 to 6076 ng/ml, liver iron concentration ranged from 1.8 to 67.97 mg/g dry weight. The amount of iron per gram liver dry weight was moderately correlated with serum ferritin values (r = 0.46). The correlation of duration of transfusion with serum ferritin (r = 0.40) and with liver iron content (r = 0.41) also indicated moderate correlation. Liver biopsy results led to changes in the management after 29/44 (66%) of the biopsies. Serum ferritin >/=2500 ng/ml predicted high liver iron content (>/=7 mg/g), with a sensitivity of 62.5% and a specificity of 77.8%.
CONCLUSION: We found a poor correlation between serum ferritin levels and liver iron content (LIC). Despite being on chelation therapy, many patients on chronic transfusion had high levels of liver iron. Measurement of LIC is highly recommended in these patients. (c) 2007 Wiley-Liss, Inc.

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Year:  2008        PMID: 17457853     DOI: 10.1002/pbc.21215

Source DB:  PubMed          Journal:  Pediatr Blood Cancer        ISSN: 1545-5009            Impact factor:   3.167


  16 in total

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5.  Decision analysis of treatment strategies in children with severe sickle cell disease.

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

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7.  Liver injury is associated with mortality in sickle cell disease.

Authors:  J J Feld; G J Kato; C Koh; T Shields; M Hildesheim; D E Kleiner; J G Taylor; N G Sandler; D Douek; V Haynes-Williams; J S Nichols; J H Hoofnagle; T Jake Liang; M T Gladwin; T Heller
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8.  Hyperferritinemia after adult allogeneic hematopoietic cell transplantation: quantification of iron burden by determining non-transferrin-bound iron.

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9.  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.

Authors:  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; Robert J Adams
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10.  Complex confounder-corrected R2* mapping for liver iron quantification with MRI.

Authors:  Diego Hernando; Rachel J Cook; Naila Qazi; Colin A Longhurst; Carol A Diamond; Scott B Reeder
Journal:  Eur Radiol       Date:  2020-08-12       Impact factor: 5.315

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