| Genotype-phenotype relationship of patients with β-thalassemia taking hydroxyurea: a 13-year experience in Iran. | |
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MedLine Citation:
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PMID: 22180324 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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We evaluated the clinical responses to hydroxyurea (HU), adverse effects, and β-globin gene variants in a large series of β-thalassemic patients over a 13-year period in Iran. The patients (n = 232) were divided into two groups: transfusion-dependent β-thalassemia patients 2 years of age and older (n = 126; Group 1), and β-thalassemia intermedia (βTI) patients without any history of blood transfusion or with a long-interval transfusion (n = 106; Group 2). In Group 1, 86 patients became transfusion-free, and 25 patients needed 1-2 transfusions per year at the end of study. All except three patients in Group 2 were completely transfusion free with a significant increase in Hb level after 1 year compared to the baseline Hb value (P < 0.0001). We did not find a significant correlation of response to HU with XmnI polymorphism or IVS II-I (G > A) mutation (P > 0.05). In our study, HU at a dose of 8-15 mg/kg/day was effective in decreasing or effecting cessation of the need for regular blood transfusion, as well as in increasing Hb levels in β-thalassemia patients, without any major side effects. Hydroxyurea may thus represent a safe alternative to blood transfusion in transfusion-dependent β-thalassemia patients, or help to increase Hb level in untransfused βTI patients. |
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Authors:
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Mehran Karimi; Sezaneh Haghpanah; Ali Farhadi; Majid Yavarian |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-12-20 |
Journal Detail:
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Title: International journal of hematology Volume: - ISSN: 1865-3774 ISO Abbreviation: - Publication Date: 2011 Dec |
Date Detail:
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Created Date: 2011-12-19 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9111627 Medline TA: Int J Hematol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran, Karimim@sums.ac.ir. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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