789 ABNORMAL METABOLISM OF β mRNA IN β+-THALASSEMIA (β-THAL)

Therapeutic approaches in patients with β-thalassemia

Beta-thalassemia (β-thal) is a congenital hemoglobinopathy explained by a decreased level (β+) or absence (βο) of β-globin gene expression. Microcytic hypochromic anemia and various clinical symptoms comprising severe anemia to clinically nonsymptomatic features. Treatment with an ordered blood transfusion and iron chelator agents can decrease transfusion iron overload that causes normal matura...

Spectrum of β-thalassemia Mutations in Iran, an Update

Abstract β-thalassemia major (β –TM) is the most common thalassemia severe phenotype among Iranians. In recent years, molecular understanding of pathogenesis of β –TM has provided a great opportunity regarding diagnostic issues. Creating comprehensive molecular databases provides highly sensitive diagnostic tools for β –TM and effective prenatal diagno...

Ineffective Erythropoiesis in β-Thalassemia

In humans, β -thalassemia dyserythropoiesis is characterized by expansion of early erythroid precursors and erythroid progenitors and then ineffective erythropoiesis. This ineffective erythropoiesis is defined as a suboptimal production of mature erythrocytes originating from a proliferating pool of immature erythroblasts. It is characterized by (1) accelerated erythroid differentiation, (2) ma...

Genomic Study in β - Thalassemia

Immunologic Abnormalities in β-Thalassemia

β-thalassemia is a hereditary hemolytic anemia associated with a decrease or complete lack of synthesis of β-globin chains as a result of inheriting the mutant gene. Currently there are more than 100 varieties of hemoglobin gene mutations, which can lead to β-thalassemia. In the world there are 60-70 thousand patients with major form of this genetic anomaly. The basic treatment for β-thalassemi...