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Non-transferrin-bound labile plasma iron and iron overload in sickle-cell disease: a comparative study between sickle-cell disease and beta-thalassemic patients.
Eur J Haematol. 2010 Jan 01; 84(1):72-8.EJ

Abstract

BACKGROUND

Blood transfusions are the standard of care in b thalassemia and transfusions are also indicated in sickle cell disease (SCD) patients with hypersplenism, recurrent vaso-occlusive crises and for stroke prevention. Iron overload caused by blood transfusions in thalassemia and in SCD may affect morbidity and mortality. Recent studies of iron overload in SCD suggest that the biologic features of SCD and the chronic inflammatory state may protect SCD patients from iron damage.

DESIGNS AND METHODS

In view of the controversy regarding the effect of iron overload in patients with SCD we studied the iron status, including non-transferrin bound iron (NTBI) and labile plasma iron (LPI) levels in a cohort of 36 SCD patients and compare the results with 43 thalassemia patients.

RESULTS

Our results indicate that none of the SCD patients had clinical symptoms of iron overload. Only two SCD patients had NTBI values in the gray zone (0.4 units) and none had positive values. By contrast, 14 patients with thalassemia major and three with thalassemia intermedia had NTBI values above 0.6, level that are in the positive pathological range. Similarly, four thalassemia patients, but only one SCD patient had positive LPI levels.

CONCLUSIONS

The parameters of iron status in SCD patients, even after frequent transfusions are different when compared to patients with thalassemia. The low NTBI and LPI levels found in patients with SCD are in keeping with the absence of clinical signs of iron overload in this disease.

Authors+Show Affiliations

Pediatric Hematology Unit and Pediatric Department B, Ha'Emek Medical Centre, Afula, Israel. koren_a@clalit.org.ilNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19732137

Citation

Koren, Ariel, et al. "Non-transferrin-bound Labile Plasma Iron and Iron Overload in Sickle-cell Disease: a Comparative Study Between Sickle-cell Disease and Beta-thalassemic Patients." European Journal of Haematology, vol. 84, no. 1, 2010, pp. 72-8.
Koren A, Fink D, Admoni O, et al. Non-transferrin-bound labile plasma iron and iron overload in sickle-cell disease: a comparative study between sickle-cell disease and beta-thalassemic patients. Eur J Haematol. 2010;84(1):72-8.
Koren, A., Fink, D., Admoni, O., Tennenbaum-Rakover, Y., & Levin, C. (2010). Non-transferrin-bound labile plasma iron and iron overload in sickle-cell disease: a comparative study between sickle-cell disease and beta-thalassemic patients. European Journal of Haematology, 84(1), 72-8. https://doi.org/10.1111/j.1600-0609.2009.01342.x
Koren A, et al. Non-transferrin-bound Labile Plasma Iron and Iron Overload in Sickle-cell Disease: a Comparative Study Between Sickle-cell Disease and Beta-thalassemic Patients. Eur J Haematol. 2010 Jan 1;84(1):72-8. PubMed PMID: 19732137.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Non-transferrin-bound labile plasma iron and iron overload in sickle-cell disease: a comparative study between sickle-cell disease and beta-thalassemic patients. AU - Koren,Ariel, AU - Fink,Daniel, AU - Admoni,Osnat, AU - Tennenbaum-Rakover,Yardena, AU - Levin,Carina, PY - 2009/9/8/entrez PY - 2009/9/8/pubmed PY - 2010/5/15/medline SP - 72 EP - 8 JF - European journal of haematology JO - Eur J Haematol VL - 84 IS - 1 N2 - BACKGROUND: Blood transfusions are the standard of care in b thalassemia and transfusions are also indicated in sickle cell disease (SCD) patients with hypersplenism, recurrent vaso-occlusive crises and for stroke prevention. Iron overload caused by blood transfusions in thalassemia and in SCD may affect morbidity and mortality. Recent studies of iron overload in SCD suggest that the biologic features of SCD and the chronic inflammatory state may protect SCD patients from iron damage. DESIGNS AND METHODS: In view of the controversy regarding the effect of iron overload in patients with SCD we studied the iron status, including non-transferrin bound iron (NTBI) and labile plasma iron (LPI) levels in a cohort of 36 SCD patients and compare the results with 43 thalassemia patients. RESULTS: Our results indicate that none of the SCD patients had clinical symptoms of iron overload. Only two SCD patients had NTBI values in the gray zone (0.4 units) and none had positive values. By contrast, 14 patients with thalassemia major and three with thalassemia intermedia had NTBI values above 0.6, level that are in the positive pathological range. Similarly, four thalassemia patients, but only one SCD patient had positive LPI levels. CONCLUSIONS: The parameters of iron status in SCD patients, even after frequent transfusions are different when compared to patients with thalassemia. The low NTBI and LPI levels found in patients with SCD are in keeping with the absence of clinical signs of iron overload in this disease. SN - 1600-0609 UR - https://www.unboundmedicine.com/medline/citation/19732137/Non_transferrin_bound_labile_plasma_iron_and_iron_overload_in_sickle_cell_disease:_a_comparative_study_between_sickle_cell_disease_and_beta_thalassemic_patients_ L2 - https://doi.org/10.1111/j.1600-0609.2009.01342.x DB - PRIME DP - Unbound Medicine ER -