Paper Information

Journal:   THE SCIENTIFIC JOURNAL OF IRANIAN BLOOD TRANSFUSION ORGANIZATION (KHOON)   FALL 2012 , Volume 9 , Number 3 (36); Page(s) 258 To 272.
 
Paper: 

APPLICATION OF STEM CELLS FOR THE TREATMENT OF HEMOGLOBINOPATHIES (REVIEW ARTICLE)

 
 
Author(s):  RAHGOZAR S.*, ENTEZAR E GHAEM M., ABEDI M., MONTAZERI F.
 
* UNIVERSITY OF ISFAHAN, HEZARJERIB STREET, ISFAHAN, IRAN
 
Abstract: 

Background and Objectives: Hemoglobinopathies are heterogenic hereditary disorders in which mutations lead to abnormal production of hemoglobin chains, or change in the normal sequence of the hemoglobin amino acids. Thalassemia and sickle cell anemia (SCA) are the most prevalent hemoglobinopathies.
Patients with the above mentioned disorders often require hyper transfusion regimens.
However, continued blood transfusion may contribute to iron overload and consequent organ deterioration, in addition to viral infections. The aim of this review article is to evaluate recent treatment programs regarding these disorders.
Materials and Methods: This paper is evaluating the newest protocols applied for the treatment of thalassemia and SCA by reviewing 51 references using Ebsco, Elsevier, Pubmed and OVID databases.
Results: Blood transfusion and medical treatments may improve the lifestyle or increase the lifespan of patients with hemoglobinopathies. However, hematopoietic stem cell transplantation (HSCT) is presented as the only curative therapy for thalassemia and SCA. On the other hand, HSCT may contribute to complications such as infertility and gonadal failure, especially in women, chronic graft-versus-host disease (GVHD), and potential secondary malignancies.
Conclusions: In spite of the limitations and complications attributed to HSCT, this method is proved to be the most effective way for treatment of hemoglobinopathies. Different cells and novel strategies used to modify transplantation are introduced in this article.

 
Keyword(s): HEMOGLOBINOPATHIES, THALASSEMIA, ANEMIA, SICKLE CELL DISORDERS, HEMATOPOIETIC STEM CELLS
 
References: 
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