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

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α:Non-α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea.

Author: Abbas NAJJARI ¹ ; Mohsen ASOURI ² ; Ladan Hosseini GOUHARI ³ ; Haleh Akhavan NIAKI ⁴ ; Amir Sasan Mozaffari NEJAD ⁵ ; Seyyedeh Masoumeh ESLAMI ⁶ ; Hassan ABOLGHASEMI ⁷ ; Ramin ATAEE ⁸ ; Abdol Ali EBRAHIMI ⁷ ; Masoumeh Rezaei MOSHAEI ⁹ ; Ali Asghar AHMADI ¹⁰
Author Information

1. Centre for collective, reflection & implementation of ideas, Undersecretary for Research and Technology, Ministry of Health and Medical Education, Tehran, Iran.
2. North Research Center, Pasteur Institute of Iran, Amol, Iran
3. Iran University of Medical Sciences, School of Paramedicine, Cellular and Molecular Research Center, Tehran, Iran.
4. Cellular and Molecular Biology Research Center, Babol University of Medical Sciences, Babol, Iran.
5. Young Researchers and Elite Club, Sari Branch, Islamic Azad University, Sari, Iran.
6. Imam Reza Hospital, Mazandaran University of Medical Sciences, Amol, Iran.
7. Shabid Beheshti University of Medical Sciences, Tehran, Iran.
8. Pharmaceutical Sciences Research Center, Mazandaran University of Medical Sciences, Sari, Iran.
9. Department of Plant Breeding and Biotechnology, Agricultural sciences & Natural Resources University of Sari, Iran.
10. North Research Center, Pasteur Institute of Iran, Amol, Iran.
Publication Type:Journal Article
Keywords: AUT-PAGE; Globin chain; Hemoglobin; Hydroxyurea; Thalassemia intermedia
From:Asian Pacific Journal of Tropical Biomedicine 2014;4(Suppl 1):S177-85
CountryChina
Language:English
Abstract:
OBJECTIVESTo elucidate the possible ways by which hydroxyurea molecules affect globin chain (α or β-like) synthesis.
METHODSA 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.
RESULTSFetal 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.
CONCLUSIONSImprovement 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.