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Inheritance of the Bantu/Benin haplotype causes less severe hemolytic and oxidative stress in sickle cell anemia patients treated with hydroxycarbamide
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نویسنده
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Okumura Jéssika V ,Silva Danilo G H ,Torres Lidiane S ,Belini-Junior Edis ,Barberino Willian M ,Oliveira Renan G ,Carrocini Gisele C S ,Gelaleti Gabriela B ,Lobo Clarisse L C ,Bonini-Domingos Claudia R
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منبع
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journal of human genetics - 2016 - دوره : 61 - شماره : 7 - صفحه:605 -611
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چکیده
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Beta s-globin gene cluster haplotypes (βs-haplotypes) can modulate the response to hydroxycarbamide (hc) treatment in sickle cell anemia (sca) patients. in brazil, the most common haplotypes are bantu and benin, and both confer a poor prognosis for patients when untreated with hc. we evaluated oxidative and hemolytic biomarkers in 48 sca patients undergoing hc treatment separated in three subgroups: bantu/bantu, bantu/benin and benin/benin haplotype. on the basis of reduced haptoglobin (hp) levels, patients with bantu/bantu haplotypes had 3.0% higher hemolysis degree when compared with those with bantu/benin haplotypes (p=0.01). the benin/benin patients had 53.6% greater lipid peroxidation index than the bantu/bantu patients (p=0.01) because of evaluated thiobarbituric acid reactive species levels. the bantu/benin subgroup had intermediate levels of hemolytic and oxidative stress markers compared with the homozygous subgroups. through strict inclusion criteria adopted, as well as consolidated and well-described hemolytic and the oxidative parameters evaluated, we suggest a haplotype-interaction response to hc treatment mediated by a ‘balance’ between the genetic factors of each haplotype studied.
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آدرس
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Sao Paulo State University (UNESP), Department of Biology, Hemoglobin and Hematological Genetic Diseases Laboratory, Brazil, Sao Paulo State University (UNESP), Department of Biology, Hemoglobin and Hematological Genetic Diseases Laboratory, Brazil, Sao Paulo State University (UNESP), Department of Biology, Hemoglobin and Hematological Genetic Diseases Laboratory, Brazil, Sao Paulo State University (UNESP), Department of Biology, Hemoglobin and Hematological Genetic Diseases Laboratory, Brazil, Sao Paulo State University (UNESP), Department of Biology, Hemoglobin and Hematological Genetic Diseases Laboratory, Brazil, Sao Paulo State University (UNESP), Department of Biology, Hemoglobin and Hematological Genetic Diseases Laboratory, Brazil, Sao Paulo State University (UNESP), Department of Biology, Hemoglobin and Hematological Genetic Diseases Laboratory, Brazil, Sao Jose do Rio Preto Medical School (FAMERP), Department of Molecular Biology, Brazil, Hematological State Institute ‘Arthur de Siqueira Cavalcanti’ (HEMORIO), Brazil, Sao Paulo State University (UNESP), Department of Biology, Hemoglobin and Hematological Genetic Diseases Laboratory, Brazil
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Authors
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