Physiopathology of Bone Modifications inβ-Thalassemia

β -thalassemia major ( β TM) or Cooley anemia is characterized by significantly reduced or absent synthesis of β -globin chains, which induces important pathologic consequences including hemolytic anemia, altered erythropoiesis, and bone marrow overstimulation. The pathogenesis of bone changes in patients with β TM is not yet completely understood. However, an unbalance in bone mineral turnover resulting from increased resorption and suppression of osteoblast activity has been detected in β TM patients. The abnormal regulation of bone metabolism may be related to hormonal and genetic factors, iron overload and iron chelation therapy, nutritional deficits, and decreased levels of physical activity. Here, we review the most recent findings on the physiopathology of bone abnormalities in β TM. Clinical presentation and radiological features of β TM-related bone changes are also discussed.