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Bone quality in beta-thalassemia intermedia: relationships with bone quantity and endocrine and hematologic variables

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Abstract

We report the first evaluation of bone quality in 70 thalassemia intermedia (TI) patients (37 males, 33 females, age 41 ± 12 years). Thirty-three patients (47%) had been transfused, 34 (49%) had been splenectomized, 39 (56%) were on iron chelation therapy, and 11 (16%) were on hydroxyurea. Mean hemoglobin was 9.2 ± 1.5 g/dl, median ferritin 537 ng/dl (range 14–4893), and mean liver iron concentration 7.6 ± 6.4 mg Fe/g dw. Fifteen patients (21%) had endocrinopathies, and 29 (41%) had vitamin D deficiency. Bone quantity (bone mineral density, BMD) and bone quality (trabecular bone score, TBS) were evaluated by densitometry. In 53/70 patients (76%), osteopathy was found (osteoporosis in 26/53, osteopenia in 27/53). BMD values were higher in the never-transfused patients and in the not-chelated group. A highly significant correlation was found between splenectomy and BMD at all the sites, with lower values in the splenectomized patients. TBS values were significantly lower in TI patients than in 65 non-thalassemic controls (1.22 vs 1.36, p < 0.01), mainly in those splenectomized and in the transfused and chelated groups (p < 0.01). TBS did not correlate with liver iron concentration values. Our data disclose the major role of non-invasive bone quality evaluation in TI patients, especially those with the worst health state, to obtain a comprehensive assessment of fracture risk. Splenectomy seems to play a major part in bone complications.

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Article Open access 01 July 2023

Enisa Shevroja, Jean-Yves Reginster, … Nicholas C. Harvey

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Acknowledgements

We thank Prof. Cass Ingerson for editing the manuscript.

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Authors and Affiliations

  1. Rare Diseases Center, Department of Medicine and Medical Specialties, Ca’ Granda Foundation IRCCS Ospedale Maggiore Policlinico, Milan, Italy

    Marina Baldini, M. D. Cappellini & G. Graziadei

  2. UO Medicina Interna, Padiglione Granelli, IRCCS Fondazione Ca’ Granda Ospedale Maggiore Policlinico, via Francesco Sforza 35, 20122, Milan, Italy

    Marina Baldini

  3. Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy

    A. Marcon, S. Seghezzi, R. Cassin & M. D. Cappellini

  4. Nuclear Medicine, Bone Metabolic Unit, Ca’ Granda Foundation IRCCS Ospedale Maggiore Policlinico, Milan, Italy

    F. M. Ulivieri

  5. Scuola di Specializzazione in Radiodiagnostica, University of Milan, Milan, Italy

    C. Messina

Authors
  1. Marina Baldini
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  2. A. Marcon
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  3. F. M. Ulivieri
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  4. S. Seghezzi
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  5. R. Cassin
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  6. C. Messina
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  7. M. D. Cappellini
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  8. G. Graziadei
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Correspondence to Marina Baldini.

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The authors declare that they have no conflict of interest.

Funding

The study was partially funded by grants of Ministero della Salute (RC 2016).

Ethical approval

All the procedures performed were in accordance with the ethical standards of the responsible research committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008. The study has been approved by the institutional research ethics committee. Informed consent was obtained from all individual participants included in the study.

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Baldini, M., Marcon, A., Ulivieri, F.M. et al. Bone quality in beta-thalassemia intermedia: relationships with bone quantity and endocrine and hematologic variables. Ann Hematol 96, 995–1003 (2017). https://doi.org/10.1007/s00277-017-2959-0

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  • DOI: https://doi.org/10.1007/s00277-017-2959-0

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