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

• Published in: Asian Pacific Journal of Tropical Biomedicine
• Main Authors: 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
• Format: Article in Journal/Newspaper
• Language: English
• Published: Wolters Kluwer Medknow Publications 2014
• Subjects: Thalassemia intermedia; Globin chain; AUT-PAGE; Hydroxyurea; Hemoglobin; Arctic medicine. Tropical medicine; RC955-962; Biology (General); QH301-705.5; Arctic; Sickle
• Online Access: https://doi.org/10.12980/APJTB.4.2014C1161; https://doaj.org/article/3b48ae89f0834cddabaa88b28e45be4d

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.