Psychological Aspects of Thalassemia Disease
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Abstract
Hemoglobin is a protein present in erythrocytes responsible for carrying oxygen throughout the body. A hemoglobin molecule has two sub-units usually indicated to as alpha and beta. Both sub-units are necessary to bind oxygen and deliver it to cells and tissues in the body. The gene that controlled the production of alpha chains is called the alpha globin gene cluster, and similarly the beta globin gene produced beta chains. Thalassemia is characterized as hemoglobinopathy, which means that it is pathophysiology that which occurs due to hemoglobin protein deficiency found in red blood cells (RBCS). More specifically, alpha and beta thalassemia are caused by gene mutations that code for the alpha and beta globin chains that make up the quaternary structure of hemoglobin. Thalassemia is a genetic disorder with mutations in the a-globin (chromosome 16) or B-globin (chromosome 11) gene, resulting in a loss of the affected globin chain but an accumulation of the non-affected one, resulting in an excess in the globin chain. As a consequence, inadequate erythropoiesis, damage to erythroid membranes and spleen obstruction may occur.The term "thalassemia" derives from Greek words (Thalassa) meaning sea and blood (Heam)The two major types of thalassemia, alpha and beta, are named after defects in these protein chains. Severe forms usually are diagnosed in early childhood and are lifelong conditions.
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