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Endocrine and bone disease in appropriately treated adult patients with beta-thalassemia major

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Abstract

With the optimization of transfusional and chelation regimens, beta-thalassemia has changed from a pediatric disease with poor life expectancy into a chronic disease. Bone demineralization is an important cause of morbidity in older patients; the etiology is multifactorial and partially unknown. We examined, cross-sectionally, 111 adult patients with beta-thalassemia major (66 females and 45 males, 32.6 ± 6 years) who were regularly transfused, sufficiently chelated and replaced for endocrine defects. Bone demineralization was detected in 92.7% of patients with different severity according to gender and site: osteopenia was the prominent finding at the femur, osteoporosis at the lumbar spine (p < 0.001), more evident in males. The femoral site was more influenced by biochemical and clinical factors; despite adequate replacement, the femoral T-score was lower in the hypogonadic group than in the eugonadic group (p = 0.047). A significant correlation was found between the bone mass and body mass index (BMI), alkaline phosphatase (ALP), and pre-transfusional Hb levels. The multivariate analysis indicated as significant regressors ALP, BMI and hypoparathyroidism (T-score, p = 0.005, 0.035, and 0.002; Z-score, 0.002, 0.009, and 0.003, respectively) at the femoral site; whereas, only ALP at the lumbar spine (p = 0.008 and 0.045 for T-and Z-scores, respectively). The statistical significance was reached more frequently by the T-score, while the Z-score seemed to be a less sensitive parameter. Despite best care facilities, bone demineralization in thalassemic patients remains a challenge. Further exploration of the relationships between bone loss and endocrine, biochemical and hematologic parameters is warranted to find effective measures to reduce the risk of fracture in this disease.

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References

  1. Borgna-Pignatti C, Cappellini MD, De Stefano P, Del Vecchio GC, Forni GL, Gamberini MR, Ghilardi R, Origa R, Piga A, Romeo MA, Zhao H, Cnaan A (2005) Survival and complications in thalassemia. Ann N Y Acad Sci 1054:40–47

    Article  CAS  PubMed  Google Scholar 

  2. Vogiatzi MG, Macklin EA, Fung EB, Vichinsky E, Olivieri N, Kwiatkowski J, Cohen A, Neufeld E, Giardina PJ (2006) Prevalence of fractures among the thalassemia syndromes in North America. Bone 38(4):571–575

    Article  CAS  PubMed  Google Scholar 

  3. Vogiatzi MG, Macklin EA, Fung EB, Cheung AM, Vichinsky E, Olivieri N, Kirby M, Kwiatkowski JL, Cunningham M, Holm IA, Lane J, Schneider R, Fleisher M, Grady RW, Peterson CC, Giardina PJ, Network Thalassemia Clinical Research (2009) Bone disease in thalassemia: a frequent and still unresolved problem. J Bone Miner Res 24(3):543–557

    Article  PubMed  Google Scholar 

  4. Cappellini M.D, Cohen A, Eleftheriou A, Piga A, Porter J, Taher A (2008) Guidelines for the clinical management of thalassemia; 2nd revised Edition. Thalassemia International Federation. http://thalassemia.org.cy

  5. Wood JC, Enriquez C, Ghugre N, Tyzka JM, Carson S, Nelson MD, Coates TD (2005) MRI R2 and R2* mapping accurately estimates hepatic iron concentration in transfusion-dependent thalassemia and sickle cell disease patients. Blood 106(4):1460–1465

    Article  CAS  PubMed  Google Scholar 

  6. Dubey AP, Parakh A, Dublish S (2008) Current trends in the management of beta thalassemia. Indian J Pediatr 75(7):739–743

    Article  CAS  PubMed  Google Scholar 

  7. Perrotta S, Cappellini MD, Bertoldo F, Servedio V, Iolascon G, D'Agruma L, Gasparini P, Siciliani MC, Iolascon A (2000) Osteoporosis in beta-thalassaemia major patients: analysis of the genetic background. Br J Haematol 111(2):461–466

    Article  CAS  PubMed  Google Scholar 

  8. Origa R, Fiumana E, Gamberini MR, Armari S, Mottes M, Sangalli A, Paglietti E, Galanello R, Borgna-Pignatti C (2005) Osteoporosis in beta-thalassemia: clinical and genetic aspects. Ann N Y Acad Sci 1054:451–456

    Article  CAS  PubMed  Google Scholar 

  9. Skordis N (2009) The labyrinth of bone disease in thalassaemia: the search for Ariadne's thread continues. Eur J Haematol 82(1):13–14

    Article  PubMed  Google Scholar 

  10. Gurevitch O, Slavin S (2006) The hematological etiology of osteoporosis. Med Hypotheses 67(4):729–735

    Article  PubMed  Google Scholar 

  11. Voskaridou E, Terpos E (2008) Pathogenesis and management of osteoporosis in thalassemia. Pediatr Endocrinol Rev 6(Suppl 1):86–93

    PubMed  Google Scholar 

  12. Salama OS, Al-Tonbary YA, Shahin RA, Eldeen OA (2006) Unbalanced bone turnover in children with beta-thalassemia. Hematology 11(3):197–202

    Article  CAS  PubMed  Google Scholar 

  13. Dresner Pollack R, Rachmilewitz E, Blumenfeld A, Idelson M, Goldfarb AW (2000) Bone mineral metabolism in adults with beta-thalassaemia major and intermedia. Br J Haematol 111(3):902–907

    Article  CAS  PubMed  Google Scholar 

  14. Voskaridou E, Kyrtsonis MC, Terpos E, Skordili M, Theodoropoulos I, Bergele A, Diamanti E, Kalovidouris A, Loutradi A, Loukopoulos D (2001) Bone resorption is increased in young adults with thalassaemia major. Br J Haematol 112(1):36–41

    Article  CAS  PubMed  Google Scholar 

  15. Morabito N, Gaudio A, Lasco A, Atteritano M, Pizzoleo MA, Cincotta M, La Rosa M, Guarino R, Meo A, Frisina N (2004) Osteoprotegerin and RANKL in the pathogenesis of thalassemia-induced osteoporosis: new pieces of the puzzle. J Bone Miner Res 19(5):722–727

    Article  CAS  PubMed  Google Scholar 

  16. Hiratsuka H, Katsuta O, Toyota N, Tsuchitani M, Akiba T, Marumo F, Umemura T (1997) Iron deposition at mineralization fronts and osteoid formation following chronic cadmium exposure in ovariectomized rats. Toxicol Appl Pharmacol 143:348–356

    Article  CAS  PubMed  Google Scholar 

  17. Mahachoklertwattana P, Sirikulchayanonta V, Chuansumrit A, Karnsombat P, Choubtum L, Sriphrapradang A, Domrongkitchaiporn S, Sirisriro R, Rajatanavin R (2003) Bone histomorphometry in children and adolescents with beta-thalassemia disease: iron-associated focal osteomalacia. J Clin Endocrinol Metab 88(8):3966–3972

    Article  CAS  PubMed  Google Scholar 

  18. Carmina E, Di Fede G, Napoli N, Renda G, Vitale G, Lo Pinto C, Bruno D, Malizia R, Rini GB (2004) Hypogonadism and hormone replacement therapy on bone mass of adult women with thalassemia major. Calcif Tissue Int 74(1):68–71

    Article  CAS  PubMed  Google Scholar 

  19. Adami S, Giannini S, Bianchi G, Sinigaglia L, Di Munno O, Fiore CE, Minisola S, Rossini M (2009) Vitamin D status and response to treatment in post-menopausal osteoporosis. Osteoporos Int 20(2):239–244

    Article  CAS  PubMed  Google Scholar 

  20. Napoli N, Carmina E, Bucchieri S, Sferrazza C, Rini GB, Di Fede G (2006) Low serum levels of 25-hydroxy vitamin D in adults affected by thalassemia major or intermedia. Bone 38(6):888–892

    Article  CAS  PubMed  Google Scholar 

  21. Angelopoulos NG, Goula A, Rombopoulos G, Kaltzidou V, Katounda E, Kaltsas D, Tolis G (2006) Hypoparathyroidism in transfusion-dependent patients with beta-thalassemia. J Bone Miner Metab 24(2):138–145

    Article  PubMed  Google Scholar 

  22. Laway BA, Goswami R, Singh N, Gupta N, Seith A (2006) Pattern of bone mineral density in patients with sporadic idiopathic hypoparathyroidism. Clin Endocrinol (Oxf) 64(4):405–409

    CAS  Google Scholar 

  23. Christoforidis A, Kazantzidou E, Tsatra I, Tsantali H, Koliakos G, Hatzipantelis E, Katzos G, Athanassiou-Metaxa M (2007) Normal lumbar bone mineral density in optimally treated children and young adolescents with beta-thalassaemia major. Hormones (Athens) 6(4):334–340

    Google Scholar 

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Authors and Affiliations

  1. Internal Medicine 1 A, Department of Internal Medicine, University of Milan, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, via F. Sforza 35, 20122, Milan, Italy

    Marina Baldini, Alessia Marcon, Alessandra Orsatti, Benedetta Tampieri, Laura Zanaboni & Maria Domenica Cappellini

  2. Audiology Unit, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, via F. Sforza 35, 20122, Milan, Italy

    Stella Forti

  3. Nuclear Medicine, Bone Metabolic Unit, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, via F. Sforza 35, 20122, Milan, Italy

    Fabio Massimo Ulivieri

  4. Internal Medicine 1 B, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, via F. Sforza 35, 20122, Milan, Italy

    Lorena Airaghi

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  1. Marina Baldini
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  2. Stella Forti
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  3. Alessia Marcon
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  4. Fabio Massimo Ulivieri
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  5. Alessandra Orsatti
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  6. Benedetta Tampieri
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  7. Lorena Airaghi
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  8. Laura Zanaboni
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  9. Maria Domenica Cappellini
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Correspondence to Marina Baldini.

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Baldini, M., Forti, S., Marcon, A. et al. Endocrine and bone disease in appropriately treated adult patients with beta-thalassemia major. Ann Hematol 89, 1207–1213 (2010). https://doi.org/10.1007/s00277-010-1007-0

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  • DOI: https://doi.org/10.1007/s00277-010-1007-0

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