title: α:Non-α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea.
abstract: 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.
authors.affiliation: Centre for collective, reflection & implementation of ideas, Undersecretary for Research and Technology, Ministry of Health and Medical Education, Tehran, Iran.
authors.forename: Abbas
authors.initials: A
authors.lastname: Najjari
authors.affiliation: North Research Center, Pasteur Institute of Iran, Amol, Iran ; 17th Shahrivar Hospital, Mazandaran University of Medical Sciences, Amol, Iran.
authors.forename: Mohsen
authors.initials: M
authors.lastname: Asouri
authors.affiliation: Iran University of Medical Sciences, School of Paramedicine, Cellular and Molecular Research Center, Tehran, Iran.
authors.forename: Ladan Hosseini
authors.initials: LH
authors.lastname: Gouhari
authors.affiliation: Cellular and Molecular Biology Research Center, Babol University of Medical Sciences, Babol, Iran.
authors.forename: Haleh Akhavan
authors.initials: HA
authors.lastname: Niaki
authors.affiliation: Young Researchers and Elite Club, Sari Branch, Islamic Azad University, Sari, Iran.
authors.forename: Amir Sasan Mozaffari
authors.initials: AS
authors.lastname: Nejad
authors.affiliation: Imam Reza Hospital, Mazandaran University of Medical Sciences, Amol, Iran.
authors.forename: Seyyedeh Masoumeh
authors.initials: SM
authors.lastname: Eslami
authors.affiliation: Shabid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolghasemi
authors.affiliation: Pharmaceutical Sciences Research Center, Mazandaran University of Medical Sciences, Sari, Iran.
authors.forename: Ramin
authors.initials: R
authors.lastname: Ataee
authors.affiliation: Shabid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Abdol Ali
authors.initials: AA
authors.lastname: Ebrahimi
authors.affiliation: Department of Plant Breeding and Biotechnology, Agricultural sciences & Natural Resources University of Sari, Iran.
authors.forename: Masoumeh Rezaei
authors.initials: MR
authors.lastname: Moshaei
authors.affiliation: North Research Center, Pasteur Institute of Iran, Amol, Iran.
authors.forename: Ali Asghar
authors.initials: AA
authors.lastname: Ahmadi
keywords: AUT-PAGE; Globin chain; Hemoglobin; Hydroxyurea; Thalassemia intermedia