Angiogenesis, Inflammation & Therapeutics | Online ISSN  2207-872X
RESEARCH ARTICLE   (Open Access)

Association Between Low Bone Mineral Density in Hemophilia Patients and Musculoskeletal Function Impairments

Rasha Ibrahim Salman 1* , Khalid Mahdi Salih 1*, Nidal Karim Al-Rahal 2*

+ Author Affiliations

Journal of Angiotherapy 8(2) 1-8 https://doi.org/10.25163/angiotherapy.829471

Submitted: 01 November 2023  Revised: 04 February 2024  Published: 15 February 2024 

Abstract

Background: Hemophilia A (HA) and B (HB) are inherited bleeding disorders due to the partial or total deficiency of coagulation factor (F) VIII or FIX, respectively. It is associated with low bone mineral density (BMD). Method: The current study was conducted on sixty-eight Iraqi patients with hemophilia to assess BMD using a DEXA scan enrolled from the National Center of Hematology / Mustansiriyah University from July 2020 to September 2021. Various medical history characteristics were recorded as (age, type of hemophilia, severity, number and site of bleeding, smoking, and alcohol intake) as well as Functional Independence Score in Haemophilia (FISH) was used to assess musculoskeletal functions and Hepatitis C virus (HCV)/Human immunodeficiency virus (HIV) seropositivity were recorded. Results: The results found that only  39.7% of patients have positive DEXA scans characterized by low BMD (abbreviated as DX+) and 60.3 % with normal BMD (abbreviated as DX0). The average Functional Independence (FISH) score in DX+ patients is significantly lower than those in the DX0 group, and about 33.3% of patients in the DX+ group have limitation of movement (LOM), which is significantly higher than of patients in DX0 group and 70.4% of patients in DX+ group are presented with seropositive viral infection, which is significantly higher than 39% of patients in DX0 group.  Conclusion: It can be concluded that hemophilic patients with seropositive viral infection and severe LOM may be at higher risk of developing low BMD.

Keywords: Hemophilia, Bone Mineral Density, DEXA Scan, Functional Independence Score, Musculoskeletal Function

References

Adler, R. A. (2011). Osteoporosis in men: what has changed?. Current osteoporosis reports, 9(1), 31-35.

Ahmed, S. G., Ibrahim, U. A and Hassan, A. W. (2007). Adequacy and pattern of blood donations in north–eastern Nigeria: the implications for blood safety. Annals of Tropical Medicine & Parasitology; 101(8): 725-731.

Alli, N., Vaughan, J., Louw, S., Schapkaitz, E and Mahlangu, J (2018). Inherited bleeding disorders. South African Medical Journal, 108(1): 9-15.

Anagnostis P, Karras S, Paschou SA and Goulis DG (2015). Haemophilia A and B as a cause for secondary osteoporosis and increased fracture risk. Blood Coagul Fibrinolysis; 26:599-603.

 Anagnostis, P., Vakalopoulou, S., Slavakis, A., Charizopoulou, M., Kazantzidou, E., Chrysopoulou, T and Garipidou, V (2012). Reduced bone mineral density in patients with haemophilia A and B in Northern Greece. Thrombosis and haemostasis; 107(03): 545-551.

Ashritha A, Delhi Kumar CG, Sahoo J and Nalini P (2019). Evaluation of Bone Mineral Density in Children with Hemophilia: An Observational Case-Control Study. J Pediatr Hematol Oncol; 41 (7): 511-514.

Ayyash H, Abu Mustafa AM and Alhabbibi B.(2020). Evaluation of Nutritional Status among Adult Hemophilia Patients Enrolled In European Gaza Hospital in Gaza strip, Palestine. J Clin Med Res; 2:1-14.

Balkan C, Kavakli K, and Karapinar D (2005). Ilipsoas haemorrhage in patients with haemophilia: results from one Centre. Haemophilia; 11: 463-7.

Beyer R, Ingerslev J, Sørensen B (2010 a). Muscle bleeds in professional athletes-diagnosis, classification, treatment and potential impact in patients with haemophilia. Haemophilia; 16: 858-65.

Bordbar, M., Olyaeinezhad, S., Saki, F and Haghpanah, S. (2020). Prevalence of Low Bone Mass in Patients with Hemophilia and Its Related Ractors in Southern Iran. Journal of Comprehensive Pediatrics; 11(4).

Christoforidis A, Economou M, Papadopoulou E, Kazantzidou E, Gompakis N, and Athanassiou-Metaxa M (2010). Bone status of children with hemophilia A assessed with quantitative ultrasound sonography (QUS) and dual energy X-ray absorptiometry (DXA). J Pediatr Hematol Oncol; 32: e259-63.

Dagli, M., Kutlucan, A., Abusoglu, S., Basturk, A., Sozen, M., Kutlucan, L and Yilmaz, F (2018). Evaluation of bone mineral density (BMD) and indicators of bone turnover in patients with hemophilia. Bosnian Journal of Basic Medical Sciences; 18(2): 206.

Dauty M, Sigaud M, Trossaërt M, Fressinaud E, Letenneur J, and Dubois C (2007). Iliopsoas hematoma in patients with hemophilia: a single-center study. Joint Bone Spine; 74: 179-83      

De Kleijn, P., Sluiter, D., Vogely, H. C., Lindeman, E and Fischer, K (2014). Long-term outcome of multiple joint procedures in haemophilia. Haemophilia; 20(2):276-281.

Ekinci O, Demircioglu S, Dogan A, Merter M, Yildiz S and Demir C (2019). Decreased bone mineral density and associated factors in severe haemophilia A patients: A case-control study. Haemophilia. 2019; 25 (5): e315-e321. 

Eldash HH, Atwa ZT and Saad MA (2017). Vitamin D deficiency and osteoporosis in hemophilic children: an intermingled comorbidity. Blood Coagul Fibrinolysis; 28:14-18.

Gamal Andrawes N, Hashem Fayek M, Salah El-Din N and Atef Mostafa R (2020). 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; 26 (2): 325-332.

Gay ND, Lee SC, Liel MS, Sochacki P, Recht M and Taylor JA (2015). Increased fracture rates in people with haemophilia: a 10-year single institution retrospective analysis. Br J Haematol; 170 (4): 584-6. 

Gebetsberger J, Schirmer M, Wurzer WJ and Streif W (2022) Low Bone Mineral Density in Hemophiliacs. Front Med; 9: 794456.

Gerstner G, Damiano ML, Tom A, Worman C, Schultz W, Recht M and Stopeck AT (2009). Prevalence and risk factors associated with decreased bone mineral density in patients with haemophilia. Haemophilia; 15 (2): 559-65. 

Gerstner G, et al (2009). Prevalence and risk factors associated with decreased bone mineral density in patients with haemophilia. Haemophilia; 15: 559–565

Gerstner, G., Damiano, M. L., Tom, A., Worman, C., Schultz, W., Recht, M., and Stopeck, A. T (2009). Prevalence and risk factors associated with decreased bone mineral density in patients with Haemophilia. Haemophilia; 15 (2): 559-565.

Gurcay E, Eksioglu E, Ezer U, Tuncay R, Cakci A. Functional disability in children with hemophilic arthropathy. Rheumatol Int 2006; 26:1031-5

Hassan TH, Badr MA, El-Gerby KM. Correlation between musculoskeletal function and radiological joint scores in haemophilia A adolescents. Haemophilia 2011; 17:920-5.

Iorio A, Fabbriciani G, Marcucci M, Brozzetti M and Filipponi P (2010). Bone mineral density in haemophilia patients. A meta-analysis. Thromb Haemost; 103 (3): 596-603.

Kadhim KA-R, Al-Lami FH, and Baldawi KH (2019).  Epidemiological Profile of Hemophilia in Baghdad-Iraq. Inquiry; 56: 1-8.

Katharina Holstein, Leonora Witt, Tim Rolvien, Michael Amling, Florian Barvencik, Anna Matysiak and Florian Langer (2020). Impact of Hepatitis C Infection on Bone Microstructure of Patients with Hemophilia, Hamostaseologie; 40(S 01): S33-S5.

Katsarou, O., Terpos, E., Chatzismalis, P., Provelengios, S., Adraktas, T., Hadjidakis, D., and Karafoulidou, A (2010). Increased bone resorption is implicated in the pathogenesis of bone loss in hemophiliacs: correlations with hemophilic arthropathy and HIV infection. Annals of hematology; 89(1): 67-74.

Kempton CL, Antoniucci DM and Rodriguez-Merchan EC (2015). Bone health in persons with Haemophilia. Haemophilia; 21:568-77.

Klintman, J., Akesson, K. E., Holme, P. A and Fischer, K. (2022). Bone mineral density in haemophilia–a multicenter study evaluating the impact of different replacement regimens. Haemophilia; 28(2): 239-246.

Kovacs CS (2008). Hemophilia, low bone mass, and osteopenia/osteoporosis. Transfus Apher Sci; 38 (1): 33-40.

Linari S, Melchiorre D, Pieri L, Tofani L, Fanelli A, Brogi M and Castaman G (2020). Low bone mass and hypovitaminosis D in haemophilia: A single-Centre study in patients with severe and moderate haemophilia A and B. Haemophilia; 26 (5): 898-906.

Makris, M and Preston, F. E (1993). Chronic hepatitis in haemophilia. Blood reviews; 7(4): 243-250.

Mansouritorghabeh H (2015). Clinical and Laboratory Approaches to Hemophilia A. Iran J Med Sci; 40 (3): 194-205.

Martineau P, Morgan SL and Leslie WD (2021). Bone mineral densitometry reporting: pearls and pitfalls. Canadian Association of Radiologists Journal; 72 (3): 490-504.

Mehta P, Reddivari AKR. Hemophilia. [Updated 2022 Jun 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 .

Nair AP, Jijina F, Ghosh K, Madkaikar M, Shrikhande M and Nema M (2007). Osteoporosis in young haemophiliacs from western India. Am J Hematol; 82: 453-457.

Nassiri TM, Lak M, Karimi K, Managhchi M, Samimi-Rad K and Abdollahi A (2008). Seroprevalence of human immunodeficiency virus (HIV) and hepatitis C infection in hemophilic patients in Iran. Iran J Pathol; 3: 119-24.

Paschou SA, Anagnostis P, Karras S, Annweiler C, Vakalopoulou S, Garipidou V and Goulis DG (2014). Bone mineral density in men and children with haemophilia A and B: a systematic review and meta-analysis. Osteoporos Int; 25 (10): 2399-407.

Peyvandi F, Garagiola I, Young G (2016). The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet; 388(10040):187-97

Poonnoose PM, Thomas R, Keshava SN, Cherian RS, Padankatti S, Pazani D, Kavitha ML, Devadarasini M, Bhattacharji S, Viswabandya A, John JA, Macaden AS, Mathews V and Srivastava A (2007). Psychometric analysis of the Functional Independence Score in Haemophilia (FISH). Haemophilia; 13 (5): 620-6.

Roushan N, Meysamie A, Managhchi M, Esmaili J and Dormohammadi T (2014).Bone mineral density in hemophilia patients. Indian J Hematol Blood Transfus; 30(4):351-355.

Roushan N, Meysamie A, Managhchi M, Esmaili J, and Dormohammadi T (2014). Bone mineral density in hemophilia patients. Indian J Hematol Blood Transfus; 30: 351-5.

Serdar Sahin, Sevil Sadri, Zafer Baslar, and Muhlis Cem Ar (2019). Osteoporosis in Patients with Hemophilia: Single-Center Results from a Middle-Income Country, Clinical and Applied Thrombosis/Hemostasis; 25: 1-6.

Shamoon, R. P. (2017). Magnitude of arthropathy in patients with hemophilia: A single-center experience. Iraqi Journal of Hematology; 6(2): 78

Sossa Melo CL, Wandurraga EA, Peña AM, Jiménez SI, Salazar LA, Ochoa ME, Luna-Gonzalez ML, Ortiz ML, Morales K, Ayala-Castillo M, Reyes DL and Chalela CM (2018). Low bone mineral density and associated factors in patients with haemophilia in Colombia. Haemophilia; 24 (4): e222-e229. 

Tlacuilo-Parra, A., Villela-Rodriguez, J., Garibaldi-Covarrubias, R., Soto-Padilla, J and  Orozco-Alcala, J. (2010). Functional independence score in hemophilia: A cross-sectional study assessment of Mexican children. Pediatric blood & cancer; 54(3): 394-397

Tuan SH, Hu LY, Sun SF, Huang WY, Chen GB, Li MH and Liou IH (2019). Risk of osteoporotic fractures as a consequence of haemophilia: A nationwide population-based cohort study. Haemophilia; 25 (5): 876-884.

Unal K., H. D., Comert Ozkan, M., Atilla, F. D., Demirci, Z., Soyer, N., Yildirim Simsir, I., Omur, O., Capaci, K., Saydam, G and Sahin, F. (2017). Evaluation of bone mineral density and related parameters in patients with haemophilia: a single center cross-sectional study. American journal of blood research; 7(5): 59–66.

 Valentino, L. A., Hakobyan, N., Rodriguez, N and Hoots, W. K (2007). Pathogenesis of haemophilic synovitis: experimental studies on blood-induced joint damage. Haemophilia; 13:10-13.

Wallny, T. A., Scholz, D. T., Oldenburg, J., Nicolay, C., Ezziddin, S., Pennekamp, P. H and Kraft, C. N (2007). Osteoporosis in haemophilia–an underestimatedcomorbidity? Haemophilia; 13(1): 79-84.

World Federation of Hemophilia (2017). The annual global survey 2017.

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