α:Non–α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea
Objective: To elucidate the possible ways by which hydroxyurea molecules affect globin chain (α or β-like) synthesis. Methods: A total of 23 thalassemia intermedia patients (13 male and 10 female) aged between 5 and 26 years were treated for five months with 15 mg/(kg·day) of hydroxyurea. Hemoglobin...
Published in: | Asian Pacific Journal of Tropical Biomedicine |
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Wolters Kluwer Medknow Publications
2014
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Online Access: | https://doi.org/10.12980/APJTB.4.2014C1161 https://doaj.org/article/3b48ae89f0834cddabaa88b28e45be4d |
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ftdoajarticles:oai:doaj.org/article:3b48ae89f0834cddabaa88b28e45be4d 2023-05-15T15:07:07+02:00 α:Non–α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea Abbas Najjari Mohsen Asouri Ladan Hosseini Gouhari Haleh Akhavan Niaki Amir Sasan Mozaffari Nejad Seyyedeh Masoumeh Eslami Hassan Abolghasemi Ramin Ataee Abdol Ali Ebrahimi Masoumeh Rezaei Moshaei Ali Asghar Ahmadi 2014-05-01T00:00:00Z https://doi.org/10.12980/APJTB.4.2014C1161 https://doaj.org/article/3b48ae89f0834cddabaa88b28e45be4d EN eng Wolters Kluwer Medknow Publications http://www.sciencedirect.com/science/article/pii/S2221169115302641 https://doaj.org/toc/2221-1691 2221-1691 doi:10.12980/APJTB.4.2014C1161 https://doaj.org/article/3b48ae89f0834cddabaa88b28e45be4d Asian Pacific Journal of Tropical Biomedicine, Vol 4, Iss S1, Pp S177-S185 (2014) Thalassemia intermedia Globin chain AUT-PAGE Hydroxyurea Hemoglobin Arctic medicine. Tropical medicine RC955-962 Biology (General) QH301-705.5 article 2014 ftdoajarticles https://doi.org/10.12980/APJTB.4.2014C1161 2022-12-31T00:36:17Z Objective: To elucidate the possible ways by which hydroxyurea molecules affect globin chain (α or β-like) synthesis. Methods: A total of 23 thalassemia intermedia patients (13 male and 10 female) aged between 5 and 26 years were treated for five months with 15 mg/(kg·day) of hydroxyurea. Hemoglobins electrophoresis and globin chain electrophoresis was performed on each sample at different time points before and during the treatment. Results: Fetal hemoglobin increased significantly in most patients and average episodes of transfusion decreased. Both Gγ and Aγ-globin chains increased significantly and α-globin:Nonα-globin chain as well as Gγ-globin:Aγ globin chains ratios decreased. Conclusions: Improvement in α:non-α ratio and consequent decrease of free α-globin chain might be the cause of beneficial effects of hydroxyurea therapy. Two patients who felt better didn't show significant increase in their fetal hemoglobin level, and this is in contradiction with the hypothesis claiming that the HbF level increase is the cause of such therapeutic effect. In spite of the unclear mechanism of action of this drug, hydroxyurea therapy had noticeable impacts on thalassemia intermedia and also sickle cell disease and even patients suffering from thalassemia major. Article in Journal/Newspaper Arctic Directory of Open Access Journals: DOAJ Articles Arctic Sickle ENVELOPE(-66.783,-66.783,-68.867,-68.867) Asian Pacific Journal of Tropical Biomedicine 4 S177 S185 |
institution |
Open Polar |
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Directory of Open Access Journals: DOAJ Articles |
op_collection_id |
ftdoajarticles |
language |
English |
topic |
Thalassemia intermedia Globin chain AUT-PAGE Hydroxyurea Hemoglobin Arctic medicine. Tropical medicine RC955-962 Biology (General) QH301-705.5 |
spellingShingle |
Thalassemia intermedia Globin chain AUT-PAGE Hydroxyurea Hemoglobin Arctic medicine. Tropical medicine RC955-962 Biology (General) QH301-705.5 Abbas Najjari Mohsen Asouri Ladan Hosseini Gouhari Haleh Akhavan Niaki Amir Sasan Mozaffari Nejad Seyyedeh Masoumeh Eslami Hassan Abolghasemi Ramin Ataee Abdol Ali Ebrahimi Masoumeh Rezaei Moshaei Ali Asghar Ahmadi α:Non–α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea |
topic_facet |
Thalassemia intermedia Globin chain AUT-PAGE Hydroxyurea Hemoglobin Arctic medicine. Tropical medicine RC955-962 Biology (General) QH301-705.5 |
description |
Objective: To elucidate the possible ways by which hydroxyurea molecules affect globin chain (α or β-like) synthesis. Methods: A total of 23 thalassemia intermedia patients (13 male and 10 female) aged between 5 and 26 years were treated for five months with 15 mg/(kg·day) of hydroxyurea. Hemoglobins electrophoresis and globin chain electrophoresis was performed on each sample at different time points before and during the treatment. Results: Fetal hemoglobin increased significantly in most patients and average episodes of transfusion decreased. Both Gγ and Aγ-globin chains increased significantly and α-globin:Nonα-globin chain as well as Gγ-globin:Aγ globin chains ratios decreased. Conclusions: Improvement in α:non-α ratio and consequent decrease of free α-globin chain might be the cause of beneficial effects of hydroxyurea therapy. Two patients who felt better didn't show significant increase in their fetal hemoglobin level, and this is in contradiction with the hypothesis claiming that the HbF level increase is the cause of such therapeutic effect. In spite of the unclear mechanism of action of this drug, hydroxyurea therapy had noticeable impacts on thalassemia intermedia and also sickle cell disease and even patients suffering from thalassemia major. |
format |
Article in Journal/Newspaper |
author |
Abbas Najjari Mohsen Asouri Ladan Hosseini Gouhari Haleh Akhavan Niaki Amir Sasan Mozaffari Nejad Seyyedeh Masoumeh Eslami Hassan Abolghasemi Ramin Ataee Abdol Ali Ebrahimi Masoumeh Rezaei Moshaei Ali Asghar Ahmadi |
author_facet |
Abbas Najjari Mohsen Asouri Ladan Hosseini Gouhari Haleh Akhavan Niaki Amir Sasan Mozaffari Nejad Seyyedeh Masoumeh Eslami Hassan Abolghasemi Ramin Ataee Abdol Ali Ebrahimi Masoumeh Rezaei Moshaei Ali Asghar Ahmadi |
author_sort |
Abbas Najjari |
title |
α:Non–α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea |
title_short |
α:Non–α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea |
title_full |
α:Non–α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea |
title_fullStr |
α:Non–α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea |
title_full_unstemmed |
α:Non–α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea |
title_sort |
α:non–α and gγ:aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea |
publisher |
Wolters Kluwer Medknow Publications |
publishDate |
2014 |
url |
https://doi.org/10.12980/APJTB.4.2014C1161 https://doaj.org/article/3b48ae89f0834cddabaa88b28e45be4d |
long_lat |
ENVELOPE(-66.783,-66.783,-68.867,-68.867) |
geographic |
Arctic Sickle |
geographic_facet |
Arctic Sickle |
genre |
Arctic |
genre_facet |
Arctic |
op_source |
Asian Pacific Journal of Tropical Biomedicine, Vol 4, Iss S1, Pp S177-S185 (2014) |
op_relation |
http://www.sciencedirect.com/science/article/pii/S2221169115302641 https://doaj.org/toc/2221-1691 2221-1691 doi:10.12980/APJTB.4.2014C1161 https://doaj.org/article/3b48ae89f0834cddabaa88b28e45be4d |
op_doi |
https://doi.org/10.12980/APJTB.4.2014C1161 |
container_title |
Asian Pacific Journal of Tropical Biomedicine |
container_volume |
4 |
container_start_page |
S177 |
op_container_end_page |
S185 |
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1766338681872842752 |