Prospective study of histomorphometry, biochemical bone markers and bone densitometric response to pamidronate in β-thalassaemia presenting with osteopenia-osteoporosis syndrome.

Abstract

This study aimed to evaluate bone remodelling disorders in thalassaemia by using pamidronate (PD) infusion with or without hormone replacement therapy (HRT) as a diagnostic-therapeutic tool. In this prospective study, 24 adult thalassaemia major (TM) and 10 thalassaemia intermedia (TI) patients received either PD and HRT or HRT only (controls) for 3 years. Eugonadal patients with TI had PD only. Bone remodelling was assessed by dual energy X ray absorptiometry (DXA scan), type 1-collagen biochemical bone markers (BBM) and histomorphometry of iliac crest biopsy before and after PD. As a group, thalassaemics had a significant improvement in spinal and femoral bone mineral density Z scores following PD (P < 0·01) compared to the controls. Although BBM were comparable pre-therapy, they were significantly lower in the PD cohort (P < 0·001) compared to the control group. All patients had osteopenia, diminished osteoid formation and bone volume on histomorphometry pre-therapy with high turnover bone disease (HTO) in TM and low-turnover disease (LTO) in TI. In TM, bone volume improved significantly, whereas TI patients showed little or no response to PD. In conclusion, histomorphometry data suggest that TM patients have a distinct pathology of high turnover bone disease compared to TI patients, who have low-turnover disease.

• Authors: Ratna Chatterjee, Farrukh T Shah, Bernard A Davis, Margaret Byers, Dev Sooranna, Rekha Bajoria, Jean Pringle, John B Porter
• Journal: British journal of haematology (Br J Haematol) Vol. 159 Issue 4 Pg. 462-71 (Nov 2012) ISSN: 1365-2141 [Electronic] England
• PMID: 22966867 (Publication Type: Clinical Trial, Journal Article)
• Copyright: © 2012 Blackwell Publishing Ltd.

Chemical References

• Biomarkers
• Diphosphonates
• Pamidronate

Topics

• Adolescent
• Adult
• Biomarkers (analysis, blood)
• Bone Density (drug effects)
• Bone Diseases, Metabolic (blood, drug therapy, pathology)
• Bone Remodeling (drug effects)
• Bone Resorption (blood, drug therapy, pathology)
• Bone and Bones (drug effects, pathology)
• Case-Control Studies
• Densitometry
• Diphosphonates (therapeutic use)
• Female
• Humans
• Male
• Osteoporosis (blood, drug therapy, pathology)
• Pamidronate
• Prospective Studies
• Syndrome
• Young Adult
• beta-Thalassemia (blood, drug therapy, pathology)