α:Non-α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea
10.12980/APJTB.4.2014C1161
- Author:
Najjari Abbas
;
Asouri Mohsen
;
Gouhari Hosseini Ladan
;
Niaki Akhavan Haleh
;
Eslami Masoumeh Seyyedeh
;
Abolghasemi Hassan
;
Ataee Ramin
;
Ebrahimi Ali Abdol
;
Moshaei Rezaei Masoumeh
;
Ahmadi Asghar Ali
- Publication Type:Journal Article
- Keywords:
Thalassemia intermedia;
Globin chain;
AUT-PAGE;
Hydroxyurea;
Hemoglobin
- From:Asian Pacific Journal of Tropical Biomedicine
2014;(z1):177-185
- CountryChina
- Language:Chinese
-
Abstract:
Objectives: To elucidate the possible ways by which hydroxyurea molecules affect globin chain (αor β-like) synthesis.Methods: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. A total of 23 thalassemia intermedia patients (13 male and 10 female) aged between Results: Fetal hemoglobin increased significantly in most patients and average episodes of transfusion decreased. Both Gγ and Aγ-globin chains increased significantly andα-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.
