مروری بر تالاسمی و عوارض ناشی از آن

Thalassemia is a highly progressive hemolytic anemia with different levels of complexity in patients. In thalassemia, reduced level of synthesis of hemoglobin chains results in an imbalanced production of alpha and beta globin chains, and sedimentation of unpaired chains inside red blood cells is the beginning of complications among thalassemia patients. Nowadays compatible blood transfusion is the main approach in treatment of the disease, however, major problems of the patients, results from this treatment, and the most pronounced one is iron overload, which influences the function of different organs including cardiovascular, endocrine and coagulation systems. The major causes of iron overload are non-effective erythropoiesis, frequent blood transfusion, and increased iron absorption through intestine. In order to prevent iron deposition in vital organs, blood transfusions and iron chelation therapy could significantly increase the life expectancy of thalassemia patients. Today, bone marrow transplantation, molecular identification of RBC surface antigens to find the most compatible blood for the patient, and replacing old medications with newly developed ones, are very promising in reducing the dependence of patients on blood transfusion.