Defining Pathological Iron Status in Children with Thalassemia

Abstract

The purpose of this review is to summarize the recent knowledge that has been gathered, allowing a better understanding of iron status in children with thalassemia. Children with thalassemia major typically have severe anemia during the first two stages of life and receive regular red blood cell (RBC) transfusions. The results show that individuals with thalassemia major who are overtly or inadequately transfused have significant body changes that affect physical growth retardation, hostility, weak musculature, leg muscle fitness, extramedullary development of hematopoiesis, and bone expansion, as seen in some developing countries. Cases of thalassemia intermedia occur late and have mild anemia, and cannot tolerate regular blood transfusions. Treatment for thalassemia major includes regular red blood cell transfusions, iron chelating drugs, and bone marrow surgery. In addition, spleen scanning may be necessary in some cases. Also, a bone marrow transplant is the only definitive treatment currently available. The prognosis for patients with beta-thalassemia has improved most over the past 25 years, mostly due to recent medical advances in blood transfusions, iron-chelating drugs, and bone marrow transplants. However, heart disease (heart disease) has been found to remain the leading cause of death in iron-related cases.