Clin Osteol 2024; 29(4): 132-135 | DOI: 10.36290/clo.2024.016

Hemophilia and bone tissue metabolismReview articles

Palička Vladimír; Horáčková Jana; Pavlíková Ladislava; Hyšpler Radomír
Osteocentrum, Ústav klinické biochemie a diagnostiky LF UK v Hradci Králové a FN Hradec Králové

Findings of reduced bone mineral density and osteoporosis in hemophilia patients are gradually increasing and will certainly increase with successful treatment and increasing life expectancy. Older, not very numerous studies are being replaced by large meta-analyses showing significant reduction of bone mineral density in hemophiliacs. However, it is not only the effect of increasing age that is being demonstrated, but also the direct and indirect pathobiochemical links between coagulation factor deficiency and the effect of their therapeutic administration on bone tissue metabolism. A crucial role is played by the influence of the RANK/RANKL/OPG system and the direct binding of factor VIII or IX and their complex with von Willebrand factor to its components. The influence of different therapeutic pathways in the treatment of hemophiliacs is not negligible.

Keywords: hemophilia; osteoporosis; RANK, RANKL, OPG system

Published: December 11, 2024  Show citation

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Palička V, Horáčková J, Pavlíková L, Hyšpler R. Hemophilia and bone tissue metabolism. Clinical Osteology. 2024;29(4):132-135. doi: 10.36290/clo.2024.016.
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References

  1. Lin X, Gao P, Zhang Q et al. Pathogenesis and treatment of osteoporosis in patients with hemophilia. Arch Osteoporos 2023; 18(1): 17. Dostupné z DOI: <https://doi: 10.1007/s11657-022-01203-9>. Go to original source... Go to PubMed...
  2. Gebetsberger J, Schirmer M, Wurzer WJ et al. Low Bone Mineral Density in Hemophiliacs. Front Med 2022; 9: 794456. Dostupné z DOI: <https://doi: 10.3389/fmed.2022.794456>. Go to original source... Go to PubMed...
  3. Gerstner G, Damiano ML, Tom A et al. Prevalence and risk factors associated with decreased bone mineral density in patients with haemophilia. Haemophilia 2009; 15(2): 559-565. Dostupné z DOI: <https://doi: 10.1111/j.1365-2516.2008.01963.x>. Go to original source... Go to PubMed...
  4. Khawaji M, Astermark J, Äkesson Ketal. Physical activity and joint function in adults with severe haemophilia on long-term prophylaxis. Blood Coagul Fibrinolys 2011; 22(1): 50-55. Dostupné z DOI: <https:// doi: 10.1097/MBC.0b013e32834128c6>. Go to original source... Go to PubMed...
  5. Uzuner B, Ketenci S, Durmus D et al. The frequency of sarcopenia in haemophilia patients: Effects on musculoskeletal health and functional performance. Haemophilia 2024; 30(2): 505-512. Dostupné z DOI: <https://doi: 10.1111/hae.14945>. Go to original source... Go to PubMed...
  6. Soucek O, Komrska V, Hlavka Z et al. Boys with haemophilia have low trabecular bone mineral and sarcopenia, but normal bone strength at the radius. Haemophilia 2012; 18(2): 222-228. Dostupné z DOI: <https://doi: 10.1111/j.1365-2516.2011.02616.x>. Go to original source... Go to PubMed...
  7. Andrawes NG, Fayek MH, El-Din NS. Effect of low-dose factor VIII prophylaxis therapy on bone mineral density and 25(OH) vitamin D level in children with severe haemophilia A. Haemophilia 2020; 26(2): 325-332. Dostupnéz DOI: <https://doi: 10.1111/hae.13917>. Go to original source... Go to PubMed...
  8. Anagnostis P, Vakalopoulou S, Vyzantiadis TAetal. The clinical utility of bone turnover markers in the evaluation of bone disease in patients with haemophilia A and B. Haemophilia 2014; 20(2): 268-275. Dostupné z DOI: <https://doi: 10.1111/hae.12271>. Go to original source... Go to PubMed...
  9. Kempton CL, Antun A, Antoniucci DM et al. Bone density in haemophilia: a single institutional cross-sectional study. Haemophilia 2014; 20(1): 121-128. Dostupné z DOI: <https://doi: 10.1111/hae.12240>. Go to original source... Go to PubMed...
  10. Biernat MM, Jedrzejuk D, Urbaniak-Kujda D et al. Association of bone mineral density and potential risk factors for osteoporosis in patients with severe haemophilia A. Haemophilia 2024; 30(1): 130-139. Dostupné z DOI: <https://doi: 10.1111/hae.14903>. Go to original source... Go to PubMed...
  11. Cadé M, Munoz-Garcia J, Babuty A et al. FVIII at the crossroad of coagulation, bone and immune biology: Emerging evidence of biological activities beyond hemostasis. Drug Discovery Today 2022; 27(1): 102-116. DostupnézDOI: <https://doi: 10.1016/j.drudis.2021.07.015>. Go to original source... Go to PubMed...
  12. Zhang M, Song K, Wu W. Bone mineral density in haemophilia patients: A systematic review and meta-analysis. Haemophilia 2024; 30(2): 276-285. Dostupné z DOI: <https://doi: 10.1111/hae.14951 >. Go to original source... Go to PubMed...
  13. Goldscheitter G, Recht M, Sochacki P et al. Biomarkers of bone disease in persons with haemophilia. Haemophilia 2021; 27(1): 149-155. Dostupné z DOI: <https://doi: 10.1111/hae.13986>. Go to original source... Go to PubMed...
  14. Taves S, Sun J, Livingston EW et al. Hemophilia A and B mice, but not VWF-/- mice, display bone defects in congenital development and remodelling after injury. Scientific Rep 2019; 9(1): 14428. Dostupné z DOI:<https://doi: 10.1038/s41598-019-50787-9>. Go to original source... Go to PubMed...
  15. Larson EA, Taylor JA. Factor VIII Plays a Direct Role in Osteoblast Development. Blood 2017; 130(S-1): 3661. Dostupné z DOI: <https:// doi: 10.1182/blood.V130.Suppl_1.3661.366116462024>. Go to original source...
  16. El-Mikkawy DM, Elbadawy MA, El-Ghany SMA et al. Serum Sc-lerostin Level and Bone Mineral Density in Pediatric Hemophilic Arthropathy. Ind J Pediat 2019; 86(6): 515-519. Dostupné z DOI: <https://doi: 10.1007/s12098-019-02855-1>. Go to original source... Go to PubMed...
  17. Gaudio A, Xourafa A, Rapisarda R et al. Hematological Diseases and Osteoporosis. Int J Mol Sci 2020; 21(10): 3538. Dostupné z DOI: <https://doi: 10.3390/ijms21103538>. Go to original source... Go to PubMed...
  18. Rodriquez-Merchan EC, Valentino LA. Increased bone resorption in hemophilia. Blood Rev 2019; 33(1): 6-10. Dostupné z DOI: <https:// doi: 10.1016/j.blre.2018.05.002>. Go to original source... Go to PubMed...




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