Physiopathology of Bone Modifications in β-Thalassemia

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 patien...

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Main Authors: Carlo Perisano, Emanuele Marzetti, Maria Silvia Spinelli, Cinzia Anna Maria Callà, Calogero Graci, Giulio Maccauro
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Anemia
Online Access:http://dx.doi.org/10.1155/2012/320737
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author Carlo Perisano
Emanuele Marzetti
Maria Silvia Spinelli
Cinzia Anna Maria Callà
Calogero Graci
Giulio Maccauro
author_facet Carlo Perisano
Emanuele Marzetti
Maria Silvia Spinelli
Cinzia Anna Maria Callà
Calogero Graci
Giulio Maccauro
author_sort Carlo Perisano
collection DOAJ
description 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.
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series Anemia
spelling doaj-art-dd9a323904b74945863b10f3742bb6df2025-02-03T01:22:01ZengWileyAnemia2090-12672090-12752012-01-01201210.1155/2012/320737320737Physiopathology of Bone Modifications in β-ThalassemiaCarlo Perisano0Emanuele Marzetti1Maria Silvia Spinelli2Cinzia Anna Maria Callà3Calogero Graci4Giulio Maccauro5Department of Orthopaedics and Traumatology, University Hospital Agostino Gemelli, Catholic University of the Sacred Heart School of Medicine, Largo A. Gemelli 1, 00168 Rome, ItalyDepartment of Orthopaedics and Traumatology, University Hospital Agostino Gemelli, Catholic University of the Sacred Heart School of Medicine, Largo A. Gemelli 1, 00168 Rome, ItalyDepartment of Orthopaedics and Traumatology, University Hospital Agostino Gemelli, Catholic University of the Sacred Heart School of Medicine, Largo A. Gemelli 1, 00168 Rome, ItalyDepartment of Biochemistry and Clinical Biochemistry, University Hospital Agostino Gemelli, Catholic University of the Sacred Heart School of Medicine, Largo A. Gemelli 1, 00168 Rome, ItalyDepartment of Orthopaedics and Traumatology, University Hospital Agostino Gemelli, Catholic University of the Sacred Heart School of Medicine, Largo A. Gemelli 1, 00168 Rome, ItalyDepartment of Orthopaedics and Traumatology, University Hospital Agostino Gemelli, Catholic University of the Sacred Heart School of Medicine, Largo A. Gemelli 1, 00168 Rome, Italyβ-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.http://dx.doi.org/10.1155/2012/320737
spellingShingle Carlo Perisano
Emanuele Marzetti
Maria Silvia Spinelli
Cinzia Anna Maria Callà
Calogero Graci
Giulio Maccauro
Physiopathology of Bone Modifications in β-Thalassemia
Anemia
title Physiopathology of Bone Modifications in β-Thalassemia
title_full Physiopathology of Bone Modifications in β-Thalassemia
title_fullStr Physiopathology of Bone Modifications in β-Thalassemia
title_full_unstemmed Physiopathology of Bone Modifications in β-Thalassemia
title_short Physiopathology of Bone Modifications in β-Thalassemia
title_sort physiopathology of bone modifications in β thalassemia
url http://dx.doi.org/10.1155/2012/320737
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AT cinziaannamariacalla physiopathologyofbonemodificationsinbthalassemia
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