TY - JOUR
T1 - Physiopathology of Bone Modifications in β-Thalassemia
AU - Perisano, Carlo
AU - Marzetti, Emanuele
AU - Spinelli, Maria Silvia
AU - Calla', Cinzia Anna Maria
AU - Graci, Calogero
AU - Maccauro, Giulio
PY - 2012
Y1 - 2012
N2 - β-thalassemia major (βTM) 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 βTM 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 βTM
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 βTM. Clinical presentation and radiological features of βTM-related bone changes are
also discussed.
AB - β-thalassemia major (βTM) 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 βTM 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 βTM
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 βTM. Clinical presentation and radiological features of βTM-related bone changes are
also discussed.
KW - anemia
KW - anemia
UR - http://hdl.handle.net/10807/40576
M3 - Article
SN - 2090-1267
VL - 2012
SP - 1
EP - 5
JO - Anemia
JF - Anemia
ER -