Skip to main content
Erschienen in: Annals of Hematology 8/2016

03.06.2016 | Original Article

Evaluation of bone mineral density in patients with hemoglobin H disease

verfasst von: Tahereh Zarei, Sezaneh Haghpanah, Shirin Parand, Hossein Moravej, Mohammad Hossein Dabbaghmanesh, Gholamhossein Ranjbar Omrani, Mehran Karimi

Erschienen in: Annals of Hematology | Ausgabe 8/2016

Einloggen, um Zugang zu erhalten

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.
Literatur
1.
Zurück zum Zitat 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 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.
Zurück zum Zitat 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):1185–1192CrossRefPubMed 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):1185–1192CrossRefPubMed
3.
Zurück zum Zitat Fucharoen S, Winichagoon P (2012) New updating into hemoglobinopathies. Int J Lab Hematol 34(6):559–565CrossRefPubMed Fucharoen S, Winichagoon P (2012) New updating into hemoglobinopathies. Int J Lab Hematol 34(6):559–565CrossRefPubMed
4.
Zurück zum Zitat Fucharoen S, Viprakasit V (2009) Hb H disease: clinical course and disease modifiers. ASH Education Program Book 2009(1):26–34 Fucharoen S, Viprakasit V (2009) Hb H disease: clinical course and disease modifiers. ASH Education Program Book 2009(1):26–34
5.
Zurück zum Zitat Chui DH, Fucharoen S, Chan V (2003) Hemoglobin H disease: not necessarily a benign disorder. Blood 101(3):791–800CrossRefPubMed Chui DH, Fucharoen S, Chan V (2003) Hemoglobin H disease: not necessarily a benign disorder. Blood 101(3):791–800CrossRefPubMed
6.
Zurück zum Zitat Higgs DR, Wood WG, Barton C, Weatherall DJ (1983) Clinical features and molecular analysis of acquired hemoglobin H disease. Am J Med 75(2):181–191CrossRefPubMed Higgs DR, Wood WG, Barton C, Weatherall DJ (1983) Clinical features and molecular analysis of acquired hemoglobin H disease. Am J Med 75(2):181–191CrossRefPubMed
7.
Zurück zum Zitat 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 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.
Zurück zum Zitat 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 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.
Zurück zum Zitat 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):167–180PubMed 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):167–180PubMed
10.
Zurück zum Zitat Stewart T, Ralston S (2000) Role of genetic factors in the pathogenesis of osteoporosis. J Endocrinol 166(2):235–245CrossRefPubMed Stewart T, Ralston S (2000) Role of genetic factors in the pathogenesis of osteoporosis. J Endocrinol 166(2):235–245CrossRefPubMed
11.
Zurück zum Zitat 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):53–57CrossRefPubMed 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):53–57CrossRefPubMed
13.
Zurück zum Zitat Dines D, Canale V, Arnold W (1976) Fractures in thalassemia. J Bone Joint Surg 58(5):662–666PubMed Dines D, Canale V, Arnold W (1976) Fractures in thalassemia. J Bone Joint Surg 58(5):662–666PubMed
14.
Zurück zum Zitat 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):29PubMed 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):29PubMed
15.
Zurück zum Zitat 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 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
16.
Zurück zum Zitat 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 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.
Zurück zum Zitat 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):225–242CrossRefPubMed 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):225–242CrossRefPubMed
18.
Zurück zum Zitat 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):571–575CrossRefPubMed 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):571–575CrossRefPubMed
19.
Zurück zum Zitat 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 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.
Zurück zum Zitat 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):730–737CrossRefPubMed 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):730–737CrossRefPubMed
21.
Zurück zum Zitat 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):492–497CrossRefPubMed 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):492–497CrossRefPubMed
22.
Zurück zum Zitat 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):481–484CrossRefPubMed 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):481–484CrossRefPubMed
23.
Zurück zum Zitat 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):462–466CrossRefPubMed 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):462–466CrossRefPubMed
24.
Zurück zum Zitat 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):543–557CrossRefPubMed 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):543–557CrossRefPubMed
25.
Zurück zum Zitat 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):644–649CrossRefPubMed 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):644–649CrossRefPubMed
26.
Zurück zum Zitat 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 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
Metadaten
Titel
Evaluation of bone mineral density in patients with hemoglobin H disease
verfasst von
Tahereh Zarei
Sezaneh Haghpanah
Shirin Parand
Hossein Moravej
Mohammad Hossein Dabbaghmanesh
Gholamhossein Ranjbar Omrani
Mehran Karimi
Publikationsdatum
03.06.2016
Verlag
Springer Berlin Heidelberg
Erschienen in
Annals of Hematology / Ausgabe 8/2016
Print ISSN: 0939-5555
Elektronische ISSN: 1432-0584
DOI
https://doi.org/10.1007/s00277-016-2708-9

Weitere Artikel der Ausgabe 8/2016

Annals of Hematology 8/2016 Zur Ausgabe

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.