Anemia, ineffective erythropoiesis, and hepcidin: interacting factors in abnormal iron metabolism leading to iron overload in β-thalassemia
S Gardenghi, RW Grady… - Hematology/Oncology …, 2010 - hemonc.theclinics.com
As discussed in more detail in the overview by Sankaran and Nathan elsewhere in this
issue, b-thalassemia is an inherited disorder characterized by mutations in the gene
encoding b-globin that lead to the quantitative reduction or, in the most severe cases, the
total absence of b-globin synthesis in human erythroid cells. As a consequence, a-globin
chains accumulate in excess, forming aggregates that impair erythroid cell maturation, which
ultimately leads to a chronic hemolytic anemia and ineffective
issue, b-thalassemia is an inherited disorder characterized by mutations in the gene
encoding b-globin that lead to the quantitative reduction or, in the most severe cases, the
total absence of b-globin synthesis in human erythroid cells. As a consequence, a-globin
chains accumulate in excess, forming aggregates that impair erythroid cell maturation, which
ultimately leads to a chronic hemolytic anemia and ineffective
