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Iron overload and iron-chelating therapy in hemoglobin E-beta thalassemia

Iron overload and iron-chelating therapy in hemoglobin E-beta thalassemia
Iron overload and iron-chelating therapy in hemoglobin E-beta thalassemia

Whereas hemoglobin (Hb) E-beta thalassemia is recognized as probably the most common serious hemoglobinopathy worldwide, its natural history remains poorly defined. The interaction of hemoglobin E and beta-thalassemia result in a wide spectrum of clinical disorders, some indistinguishable from thalassemia major and some milder and not transfusion-dependent. Partially as a result of this wide range of phenotypes, clear guidelines for approaches to transfusion and to iron-chelating therapy for patients with Hb E-beta thalassemia have not been developed. By contrast, data that have accumulated during the past 10 years in patients with beta-thalassemia permit a quantitative approach to the management of iron overload and provide guidelines for the control of body iron burden in individual patients treated with iron-chelating therapy. These guidelines may be applicable to patients with Hb E-beta thalassemia. Preliminary evidence from our studies of iron loading in affected patients with Hb E-beta thalassemia in Sri Lanka suggest that this disorder may be associated with variable, but accelerated, gastrointestinal iron absorption, and that the iron loading associated with chronic transfusions in patients with Hb E-beta thalassemia is similar to that observed in patients with beta-thalassemia. These data, in the only cohort of patients with Hb E-beta thalassemia to have undergone quantitative assessment of body iron burden, suggest that the principles that guide assessment of iron loading and initiation of chelating therapy in patients with beta-thalassemia may be generally applicable to those with Hb E-beta thalassemia. Further quantitative studies in both nontransfused and transfused patients will be necessary to permit firm conclusions.

Adult, Blood Transfusion, Hemoglobin E, Humans, Iron, Iron Chelating Agents, Iron Overload, Liver, Male, Practice Guidelines as Topic, beta-Thalassemia, Case Reports, Journal Article
1077-4114
593-7
Olivieri, N F
1dde697f-4dbc-40ba-b875-677d7271319f
De Silva, S
4dc2c122-bc28-45c5-ad60-262c0fe9fc8a
Premawardena, A
692bf60d-2666-4ea6-8378-2637776856e9
Sharma, S
52e01ff0-3b42-4d61-b9c4-b4bb7dc71161
Viens, A M
cc615c33-4e17-41b2-b82d-2c11569c0c34
Taylor, C M
64f6985d-401e-4bef-8676-fc4fb91881b6
Brittenham, G M
885a63b1-8db6-4d93-bc24-d2d1760343c7
Weatherall, D J
1df4e31c-565d-426e-9766-935ed0043d18
Olivieri, N F
1dde697f-4dbc-40ba-b875-677d7271319f
De Silva, S
4dc2c122-bc28-45c5-ad60-262c0fe9fc8a
Premawardena, A
692bf60d-2666-4ea6-8378-2637776856e9
Sharma, S
52e01ff0-3b42-4d61-b9c4-b4bb7dc71161
Viens, A M
cc615c33-4e17-41b2-b82d-2c11569c0c34
Taylor, C M
64f6985d-401e-4bef-8676-fc4fb91881b6
Brittenham, G M
885a63b1-8db6-4d93-bc24-d2d1760343c7
Weatherall, D J
1df4e31c-565d-426e-9766-935ed0043d18

Olivieri, N F, De Silva, S, Premawardena, A, Sharma, S, Viens, A M, Taylor, C M, Brittenham, G M and Weatherall, D J (2000) Iron overload and iron-chelating therapy in hemoglobin E-beta thalassemia. Journal of pediatric hematology/oncology, 22 (6), 593-7.

Record type: Article

Abstract

Whereas hemoglobin (Hb) E-beta thalassemia is recognized as probably the most common serious hemoglobinopathy worldwide, its natural history remains poorly defined. The interaction of hemoglobin E and beta-thalassemia result in a wide spectrum of clinical disorders, some indistinguishable from thalassemia major and some milder and not transfusion-dependent. Partially as a result of this wide range of phenotypes, clear guidelines for approaches to transfusion and to iron-chelating therapy for patients with Hb E-beta thalassemia have not been developed. By contrast, data that have accumulated during the past 10 years in patients with beta-thalassemia permit a quantitative approach to the management of iron overload and provide guidelines for the control of body iron burden in individual patients treated with iron-chelating therapy. These guidelines may be applicable to patients with Hb E-beta thalassemia. Preliminary evidence from our studies of iron loading in affected patients with Hb E-beta thalassemia in Sri Lanka suggest that this disorder may be associated with variable, but accelerated, gastrointestinal iron absorption, and that the iron loading associated with chronic transfusions in patients with Hb E-beta thalassemia is similar to that observed in patients with beta-thalassemia. These data, in the only cohort of patients with Hb E-beta thalassemia to have undergone quantitative assessment of body iron burden, suggest that the principles that guide assessment of iron loading and initiation of chelating therapy in patients with beta-thalassemia may be generally applicable to those with Hb E-beta thalassemia. Further quantitative studies in both nontransfused and transfused patients will be necessary to permit firm conclusions.

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More information

Published date: 29 December 2000
Keywords: Adult, Blood Transfusion, Hemoglobin E, Humans, Iron, Iron Chelating Agents, Iron Overload, Liver, Male, Practice Guidelines as Topic, beta-Thalassemia, Case Reports, Journal Article
Organisations: Law A, Social Sciences, Medicine, Clinical & Experimental Sciences

Identifiers

Local EPrints ID: 410471
URI: https://eprints.soton.ac.uk/id/eprint/410471
ISSN: 1077-4114
PURE UUID: 72b29da3-8ae0-472c-b02a-511e3dcf08ca

Catalogue record

Date deposited: 09 Jun 2017 08:59
Last modified: 13 Mar 2019 19:47

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