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Evaluation of bone mineral density in patients with hemoglobin H disease

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Abstract

Objectives

This study was conducted to assess bone mineral density (BMD) and bone mineral content (BMC) of patients with hemoglobin H (HbH) disease.

Methods

BMD and BMC were measured by dual energy X-ray absorptiometry of the lumbar spines and femur neck in 21 patients with Hb H disease over the age of 10 years.

An association of BMD with sex, age, hemoglobin, calcium, phosphorus, and serum ferritin level was also evaluated.

Results

Prevalence of BMD below the expected range for age in the lumbar spine and femur neck region in patients with HbH disease were 33.3 and 14.3 %, respectively. Lumbar BMD was significantly lower in the patients compared to healthy individuals (median (min-max) 0.725 (0.595–0.924) vs. 1.061 (0.645–1.238), P < 0.001)). There was no significant relationship between BMD in the lumbar and femur neck with any of the evaluated variables (P value >0.05).

Conclusion

Data regarding bone density in HbH disease is limited; osteoporosis as a common complication of β-thalassemia intermedia syndrome should be considered even in HbH which shows its prevalence is less than β-thalassemia intermedia.

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References

  1. Steinberg MH, Forget BG, Higgs D R, Nagel RL (2001) Molecular mechanisms of a-thalassemia. Disorders of hemoglobin: Genetics, pathophysiology, and clinical management. Cambridge University Press 405–430

  2. Laosombat V, Viprakasit V, Chotsampancharoen T, Wongchanchailert M, Khodchawan S, Chinchang W, Sattayasevana B (2009) Clinical features and molecular analysis in Thai patients with HbH disease. Ann Hematol 88(12):1185–1192

    Article  CAS  PubMed  Google Scholar 

  3. Fucharoen S, Winichagoon P (2012) New updating into hemoglobinopathies. Int J Lab Hematol 34(6):559–565

    Article  CAS  PubMed  Google Scholar 

  4. Fucharoen S, Viprakasit V (2009) Hb H disease: clinical course and disease modifiers. ASH Education Program Book 2009(1):26–34

    Google Scholar 

  5. Chui DH, Fucharoen S, Chan V (2003) Hemoglobin H disease: not necessarily a benign disorder. Blood 101(3):791–800

    Article  CAS  PubMed  Google Scholar 

  6. Higgs DR, Wood WG, Barton C, Weatherall DJ (1983) Clinical features and molecular analysis of acquired hemoglobin H disease. Am J Med 75(2):181–191

    Article  CAS  PubMed  Google Scholar 

  7. Organization WH (1994) Assessment of fracture risk and its application to screening for postmenopausal osteoporosis: report of a WHO study group [meeting held in Rome from 22 to 25 June 1992]. World Health Organization, Geneva

  8. Larijani B, Resch H, Bonjour J, Meybodi HA, Tehrani MM (2007) Osteoporosis in Iran, overview and management. Supplementary issue on osteoporosis, Iranian Journal of Public Health:1–13

  9. De Sanctis V, Soliman AT, Elsedfy H, Yassin M, Canatan D, Kilinc Y, Sobti P, Skordis N, Karimi M, Raiola G (2013) Osteoporosis in thalassemia major: an update and the I-CET 2013 recommendations for surveillance and treatment. Pediatr Endocrinol Rev 11(2):167–180

    PubMed  Google Scholar 

  10. Stewart T, Ralston S (2000) Role of genetic factors in the pathogenesis of osteoporosis. J Endocrinol 166(2):235–245

    Article  CAS  PubMed  Google Scholar 

  11. Di Stefano M, Chiabotto P, Roggia C, Garofalo F, Lala R, Piga A, Isaia GC (2004) Bone mass and metabolism in thalassemic children and adolescents treated with different iron-chelating drugs. J Bone Miner Metab 22(1):53–57

    Article  PubMed  Google Scholar 

  12. Toumba M, Skordis N (2010) Osteoporosis syndrome in thalassaemia major: an overview. J Osteoporos 2010:537673

    Article  PubMed  PubMed Central  Google Scholar 

  13. Dines D, Canale V, Arnold W (1976) Fractures in thalassemia. J Bone Joint Surg 58(5):662–666

    CAS  PubMed  Google Scholar 

  14. Karimi M, Ghiam AF, Hashemi A, Alinejad S, Soweid M, Kashef S (2007) Bone mineral density in beta-thalassemia major and intermedia. Indian Pediatr 44(1):29

    PubMed  Google Scholar 

  15. Pollak RD, Rachmilewitz E, Blumenfeld A, Idelson M, Goldfarb AW (2000) Bone mineral metabolism in adults with β‐thalassaemia major and intermedia. Br J Haematol 111(3):902–907

    CAS  Google Scholar 

  16. Dehbozorgian J, Moghadam M, Daryanoush S, Haghpanah S, Imani fard J, Aramesh A, Shahsavani A, Karimi M (2015) Distribution of alpha-thalassemia mutations in Iranian population. Hematology 20(6):359–62

  17. Crabtree NJ, Arabi A, Bachrach LK, Fewtrell M, Fuleihan GE-H, Kecskemethy HH, Jaworski M, Gordon CM (2014) Dual-energy X-ray absorptiometry interpretation and reporting in children and adolescents: the revised 2013 ISCD Pediatric Official Positions. J Clin Densitom 17(2):225–242

    Article  PubMed  Google Scholar 

  18. Vogiatzi M, Macklin E, Fung E, Vichinsky E, Olivieri N, Kwiatkowski J, Cohen A, Neufeld E, Giardina P (2006) Prevalence of fractures among the Thalassemia syndromes in North America. Bone 38(4):571–575

    Article  CAS  PubMed  Google Scholar 

  19. Jensen C, Tuck S, Agnew J, Koneru S, Morris R, Yardumian A, Prescott E, Hoffbrand A, Wonke B (1998) High incidence of osteoporosis in thalassaemia major. J Pediatr Endocrinol Metab 11 Suppl 3:975–7

  20. Voskaridou E, Terpos E, Spina G, Palermos J, Rahemtulla A, Loutradi A, Loukopoulos D (2003) Pamidronate is an effective treatment for osteoporosis in patients with beta‐thalassaemia. Br J Haematol 123(4):730–737

    Article  CAS  PubMed  Google Scholar 

  21. Chan Y-L, Pang L-M, Chik K-W, Cheng JC, Li C-K (2002) Patterns of bone diseases in transfusion-dependent homozygous thalassaemia major: predominance of osteoporosis and desferrioxamine-induced bone dysplasia. Pediatr Radiol 32(7):492–497

    Article  PubMed  Google Scholar 

  22. Molyvda-Athanasopoulou E, Sioundas A, Karatzas N, Aggellaki M, Pazaitou K, Vainas I (1999) Bone mineral density of patients with thalassemia major: four-year follow-up. Calcif Tissue Int 64(6):481–484

    Article  CAS  PubMed  Google Scholar 

  23. Vogiatzi MG, Autio KA, Mait JE, Schneider R, Lesser M, Giardina PJ (2005) Low bone mineral density in adolescents with β‐thalassemia. Ann N Y Acad Sci 1054(1):462–466

    Article  PubMed  Google Scholar 

  24. Vogiatzi MG, Macklin EA, Fung EB, Cheung AM, Vichinsky E, Olivieri N, Kirby M, Kwiatkowski JL, Cunningham M, Holm IA (2009) Bone disease in thalassemia: a frequent and still unresolved problem. J Bone Miner Res 24(3):543–557

    Article  PubMed  Google Scholar 

  25. Morabito N, Lasco A, Gaudio A, Crisafulli A, Di Pietro C, Meo A, Frisina N (2002) Bisphosphonates in the treatment of thalassemia-induced osteoporosis. Osteoporos Int 13(8):644–649

    Article  CAS  PubMed  Google Scholar 

  26. Taher AT, Musallam KM, Karimi M, El-Beshlawy A, Belhoul K, Daar S, Saned M-S, El-Chafic A-H, Fasulo MR, Cappellini MD (2010) Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood 115 (10):1886-1892

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Acknowledgments

This study was supported by The Shiraz University of Medical Sciences with grant number 93-01-32-8943. Also, I would like to thank Forough Saki for cooperating in the data collection.

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Correspondence to Mehran Karimi.

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Zarei, T., Haghpanah, S., Parand, S. et al. Evaluation of bone mineral density in patients with hemoglobin H disease. Ann Hematol 95, 1329–1332 (2016). https://doi.org/10.1007/s00277-016-2708-9

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  • DOI: https://doi.org/10.1007/s00277-016-2708-9

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