Abstract

β-thalassemia major (βTM) or Cooley anemia is characterized by significantly reduced or absent synthesis of β-globin chains,\r\nwhich induces important pathologic consequences including hemolytic anemia, altered erythropoiesis, and bone marrow\r\noverstimulation. The pathogenesis of bone changes in patients with βTM is not yet completely understood. However, an unbalance\r\nin bone mineral turnover resulting from increased resorption and suppression of osteoblast activity has been detected in βTM\r\npatients. The abnormal regulation of bone metabolism may be related to hormonal and genetic factors, iron overload and iron\r\nchelation therapy, nutritional deficits, and decreased levels of physical activity. Here, we review the most recent findings on the\r\nphysiopathology of bone abnormalities in βTM. Clinical presentation and radiological features of βTM-related bone changes are\r\nalso discussed.
Lingua originaleInglese
pagine (da-a)1-5
Numero di pagine5
RivistaAnemia
Volume2012
Numero di pubblicazioneN/A
DOI
Stato di pubblicazionePubblicato - 2012

All Science Journal Classification (ASJC) codes

  • Ematologia
  • Biologia Cellulare

Keywords

  • anemia

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