Diagnostic and treatment methods of skeletal symptoms of haemophilia and education of patients regarding the disease – review study
Keywordshaemophilia, arthropathy, haemophilic arthropathy, radiosynovectomy, clotting factors, education
Introduction. Haemophilia is a genetic haemorrhagic disorder that characterises with extensive and prolonged bleeding. In severe stages of the disease, joint haemorrhages may occur and eventually result in joint degeneration and haemophilic arthropathy.
Aim of the study. Summary and evaluation of diagnostic and treatment methods of skeletal problems in patients with haemophilia. Review of patients’ knowledge about the disease.
Material and methods. Review of the newest medical journal literature published on PubMed database.
Results. Ultrasonography is a non-invasive imaging method used to diagnose acute hemarthrosis and early stages of joint damage. Magnetic resonance is a standard tool in evaluation and scoring of haemophilic arthropathy. X-rays and computed tomography can also be additionally used, but are ineffective in imaging soft tissue and cartilage. Substitution of clotting factors is a standard treatment in haemophilia which reduces the frequency of bleeding. Arthrocentesis, synovectomy for example radiosynovectomy are effective in case of joint disorders. Intra-joint injections of hyaluronic acid are also effective. In advanced stages of haemophilic arthropathy joint replacement is recommended. Majority of patients with haemophilia claim that they are sufficiently educated about their disease.
Conclusion. Haemophilic arthropathy is a severe joint destructing condition that causes limited mobility, chronic pain and a reduced quality of life. Its early diagnosis and proper treatment is necessary to avoid total destruction of the affected joint. Multidisciplinary care of the patient is an approach of great importance. Education of patients about their disease and up-to-date treatment methods is necessary.
Mehta P, Reddivari AKR. Hemophilia. In: StatPearls. Treasure Island (FL): StatPearls Publishing; September 30, 2022.
Basta M, Pandya AM. Genetics, X-Linked Inheritance. In: StatPearls. Treasure Island (FL): StatPearls Publishing; May 8, 2022.
Konkle BA, Nakaya Fletcher S. Hemophilia A. In: Adam MP, Everman DB, Mirzaa GM, et al., eds. GeneReviews®. Seattle (WA): University of Washington, Seattle; September 21, 2000.
https://www.mp.pl/pacjent/hematologia/choroby/170465,hemofilia (access 13.12.2022)
Buccheri E, Avola M, Vitale N, Pavone P, Vecchio M. Haemophilic arthropathy: A narrative review on the use of intra-articular drugs for arthritis. Haemophilia. 2019;25(6):919-927. doi:10.1111/hae.13857
Zhu H, Meng Y, Tong P, Zhang S. Pathological mechanism of joint destruction in haemophilic arthropathy. Mol Biol Rep. 2021;48(1):969-974. doi:10.1007/s11033-020-06052-8
Windyga J, Lopaciuk S, Stefanska E, et al. Haemophilia in Poland. Haemophilia. 2006;12(1):52-57. doi:10.1111/j.1365-2516.2006.01188.x
Foppen W, Fischer K, van der Schaaf IC. Imaging of haemophilic arthropathy: Awareness of pitfalls and need for standardization. Haemophilia. 2017;23(5):645-647. doi:10.1111/hae.13288
Gualtierotti R, Solimeno LP, Peyvandi F. Hemophilic arthropathy: Current knowledge and future perspectives. J Thromb Haemost. 2021;19(9):2112-2121. doi:10.1111/jth.15444
Soliman M, Daruge P, Dertkigil SSJ, et al. Imaging of haemophilic arthropathy in growing joints: pitfalls in ultrasound and MRI. Haemophilia. 2017;23(5):660-672. doi:10.1111/hae.13249
Rodriguez-Merchan EC. Surgical approaches to hemophilic arthropathy. Blood Coagul Fibrinolysis. 2019;30(1S Suppl 1):S11-S13. doi:10.1097/MBC.0000000000000824
Oliveira S, Thomas S, Dos Santos CLG, Berdeguez MBT, de Sa LV, de Souza SAL. Outpatient treatment for haemophilic arthropathy with radiosynovectomy: Radiation dose to family members. Haemophilia. 2019;25(3):509-513. doi:10.1111/hae.13710
Landro Carla Daffunchio Guillermo Cambiaggi Gustavo Galatro Horacio Caviglia ME, Daffunchio C, Cambiaggi G, Galatro GG, Caviglia H. Platelet-rich plasma vs platelet-rich plasma plus hyaluronic acid for haemophilic knee arthropathy treatment. Acta Orthop Belg. 2021;87(4):705-712. doi:10.52628/87.4.15
Rodriguez-Merchan EC. Intra-articular corticosteroid injections in haemophilic arthropathy: are they recommended?. Hosp Pract (1995). 2018;46(1):1-4. doi:10.1080/21548331.2018.1410425
De la Corte-Rodriguez H, Rodriguez-Merchan EC, Alvarez-Roman T, Martin-Salces M, Garcia-Barcenilla S, Jimenez-Yuste V. Health education and empowerment in adult patients with haemophilia. Expert Rev Hematol. 2019;12(11):989-995. doi:10.1080/17474086.2019.1650640
Banchev A, Batorova A, Faganel Kotnik B, et al. A Cross-National Survey of People Living with Hemophilia: Impact on Daily Living and Patient Education in Central Europe. Patient Prefer Adherence. 2021;15:871-883. Published 2021 Apr 28. doi:10.2147/PPA.S303822
le Doré S, Grinda N, Ferré E, et al. The Hemarthrosis-Simulating Knee Model: A Useful Tool for Individualized Education in Patients with Hemophilia (GEFACET Study). J Blood Med. 2021;12:133-138. Published 2021 Mar 9. doi:10.2147/JBM.S280032
How to Cite
Copyright (c) 2023 Adrianna Gorecka, Natalia Osial
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
The periodical offers access to content in the Open Access system under the Creative Commons Attribution-NonCommercial-ShareAlike 4.0
Number of views and downloads: 165
Number of citations: 0