Immune Dysfunction In Beta Thalassemia Major Patients

Beta-thalassemia major (B-th), also known as Cooley`s anemia or homozygous B-th, is a chronic genetically determined hematological disorder characterized by ineffective erythropoiesis, peripheral hemolysis and severe anemia. Patients require regular blood transfusion leading to iron overload, in turn may determine secondary systemic hemosiderosis.Infection is considered as the second cause of mortality in Beta thalassemia major patients. There are various causes of infection including blood transfusion ,splenectomy, iron overload , and aberration of function in immune system.A number of studies revealed qualitative and quantitative defects in the production of immunoglobulins, T and B lymphocytes activities ,number and function of microphages and neutrophils including chemotaxis and phagocytosis and also aberrations of complement system.In our study we aimed to evaluate the impact of Beta thalassemia major on the function of different components of the immune system including humoral, cellular, and nonspecific immunity; and on the cytokines which are the key factors of many immunologic steps, and also the relationship between immune dysfunction and epidemiological, transfusion and chelation characteristics of these patients.This study was conducted in pediatric hematology unit of Zagazig University Hospitals during the period from January 2010 to January 2012. It included 40 children (20 non splenectomized and 20 splenectomized) with B-thalassemia major and 20 age and sex matched healthy children as a control group.