Haematologica
HOME HELP FEEDBACK TABLE OF CONTENTS ARCHIVE SUBSCRIPTIONS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Yavarian, M
Right arrow Articles by Giordano, P.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Yavarian, M
Right arrow Articles by Giordano, P.
Haematologica, Vol 89, Issue 10, 1172-1178
Copyright © 2004 by Ferrata Storti Foundation

Clinical Trial

Response to hydroxyurea treatment in Iranian transfusion-dependent beta-thalassemia patients

M Yavarian, M Karimi, E Bakker, CL Harteveld, and PC Giordano

Thalassemia Medical Center, Medical Faculty, Bandar Abbas University, Iran.

BACKGROUND AND OBJECTIVES: Hydroxyurea (HU) is known to increase gamma-globin chain expression in postnatal life. The efficacy of HU treatment in thalassemia patients is still unclear. The aim of this study was to monitor treatment of a large cohort of patients with beta-thalassemia major in order to establish the response to HU and the associated elements. DESIGN AND METHODS: HU therapy was started in 133 patients diagnosed with transfusion-dependent beta-thalassemia in 1999. The molecular background of the disease, the polymorphisms of the promoter region of the genes, the haplotype of the beta-globin gene cluster, the alpha-gene deletions and the HS2 polymorphism at the locus control region (LCR) of the beta-globin gene cluster were studied. RESULTS: We were able to classify three categories of response: a good response (61%) in patients who shifted from monthly blood transfusion dependency to a stable transfusion-free condition at an average Hb level of more than 10 gm/dL; a moderate response (23%) in patients who remained transfusion dependent but at longer intervals (6 months or more), and non response in patients who, after one year of treatment, remained at the same level of transfusion dependency. The correlations with the molecular defects were found to be secondary to the presence of the (C-->T) at -158 of the Ggamma gene (XmnI polymorphism). The T allele, in linkage to the haplotype I (+----) and to the internal beta-globin gene framework 2, was the most significant modulating factor involved. INTERPRETATION AND CONCLUSIONS: The good response to HU treatment that a significant number of southwest Iranian patients with beta-thalassemia patients had seems to correlate with particular haplotypes. This indicates that HU treatment is a sensible option for transfusion-dependent beta-thalassemia patients with a favorable molecular background.


This article has been cited by other articles:


Home page
 Evid Based Complement Alternat MedHome page
N. Bianchi, C. Zuccato, I. Lampronti, M. Borgatti, and R. Gambari
Fetal Hemoglobin Inducers from the Natural World: A Novel Approach for Identification of Drugs for the Treatment of -Thalassemia and Sickle-Cell Anemia
Evid. Based Complement. Altern. Med., December 11, 2007; (2007) nem139v1.
[Abstract] [Full Text] [PDF]

Home page
 Evid Based Complement Alternat MedHome page
A. Banerjee, S. B. Chakrabarty, S. R. Karmakar, A. Chakrabarty, S. J. Biswas, S. Haque, D. Das, S. Paul, B. Mandal, B. Naoual, et al.
Can Homeopathy Bring Additional Benefits to Thalassemic Patients on Hydroxyurea Therapy? Encouraging Results of a Preliminary Study
Evid. Based Complement. Altern. Med., October 29, 2007; (2007) nem161v1.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK TABLE OF CONTENTS ARCHIVE SUBSCRIPTIONS
Copyright © 2004 by the Ferrata Storti Foundation.