Skip to main content

Hemophilic arthropathy: clinical, radiologic, and functional evaluation: a single-center experience in a limited resource country



Hemophilia A and B are clinically indistinguishable and are heterogeneous disorders. The severity of bleeding symptoms correlates with the coagulant activity of the deficient factor. Joint bleeding initially leads to independent adverse changes in both the synovial tissue and the articular cartilage.


The aim of the present work was to evaluate hemophilic joints clinically, radiologically, and functionally in patients with hemophilic arthropathy.

Materials and methods

The study was carried out on 30 boys suffering from hemophilic arthropathy; the mean age was 10.6 ± 2.95 years. All patients were subjected to thorough history taking and local physical examination of the ‘target joint’. Functional Independence Score in Hemophilia (FISH) and the Pettersson scoring system were assessed for all patients.


The age at first hemarthrosis decreased with the severity of hemophilia, whereas the number of bleeds/year and the number of joints affected increased with the severity, and the results were statistically significant. A statistically significant positive correlation was found between the Pettersson score and both the age of the patients and the number of bleeds/year. However, a negative correlation was found with factor activity level. In contrast, the FISH score had a significant positive correlation with factor activity level.


A significant decrease in the functional ability was demonstrated on the basis of the severity of hemophilia. Both the FISH and Pettersson scoring systems are of great importance in assessing patients with hemophilic arthropathy.


  1. Jayandharan GR, Srivastava A. Hemophilia: genetics, diagnosis and treatment. Genet Syndr Gene Ther 2011; 1:1–12.

    Google Scholar 

  2. Hoffman M. A cell-based model of coagulation and the role of factor VIIa. Blood Rev 2003; 17(Suppl 1):1–5.

    Article  Google Scholar 

  3. Paul-Scott J, Robert R. Montgomery hereditary clotting factor deficiencies (bleeding disorders). In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, (editors) Nelson textbook of pediatrics. 19th ed. Philadelphia: Saunders Elsevier; 2011. 470:1699–1701.

    Chapter  Google Scholar 

  4. White GC II, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J, Factor VIII and Factor IX Subcommittee. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001; 85: 560.

    Article  CAS  Google Scholar 

  5. Rodriguez-Merchan EC, Jimenez-Yuste V, Aznar JA, Hedner U, Knobe K, Lee CA, et al. Joint protection in haemophilia. Haemophilia 2011; 17(Suppl 2): 1–23.

    Article  Google Scholar 

  6. Roosendaal G, Lafeber F. Prophylactic treatment for prevention of joint disease in hemophilia – cost versus benefit. N Engl J Med 2007; 357: 603– 605.

    Article  CAS  Google Scholar 

  7. Jansen NW, Roosendaal G, Lafeber FP. Understanding haemophilic arthropathy: an exploration of current open issues. Br J Haematol 2008; 143:632–640.

    Article  Google Scholar 

  8. Jacobson JA, Girish G, Jiang Y, Sabb BJ. Radiographic evaluation of arthritis: degenerative joint disease and variations. Radiology 2008; 248:737– 747.

    Article  Google Scholar 

  9. Pettersson H, Ahlberg A, Nilsson IM. A radiologic classification of hemophilic arthropathy. Clin Orthop Relat Res 1980; 149: 153–153159.

    Google Scholar 

  10. De Kleijn P, Heijnen L, Van Meeteren NL. Clinimetric instruments to assess functional health status in patients with haemophilia: a literature review. Haemophilia 2002; 8:419–427.

    Article  Google Scholar 

  11. Pergantou H, Matsinos G, Platokouki H, Papadopoulos A, Aronis S. An attempt to improve the clinical scale for assessment of haemophilic arthropathy in children. J Pediatr Orthop B 2009; 18:204–210.

    Article  Google Scholar 

  12. Poonnoose PM, Thomas R, Keshava SN, Cherian RS, Padankatti S, Pazani D et al. Psychometric analysis of the Functional Independence Score in Haemophilia (FISH). Haemophilia 2007; 13:620–626.

    Article  CAS  Google Scholar 

  13. Jansen NW, Roosendaal G, Lundin B, Heijnen L, Mauser-Bunschoten E, Bijlsma JW, et al. The combination of the biomarkers urinary C-terminal telopeptide of type II collagen, serum cartilage oligomeric matrix protein, and serum chondroitin sulfate 846 reflects cartilage damage in hemophilic arthropathy. Arthritis Rheum 2009; 60:290–298.

    Article  CAS  Google Scholar 

  14. Valentino LA, Taylor A. Hemophilia Clinical Consults: hemophilic arthropathy, reduced bone density and preventive strategies. Clinical Consults 2011; 1:1.

    Google Scholar 

  15. Stephensen D, Tait RC, Brodie N, Collins P, Cheal R, Keeling D, et al. Changing patterns of bleeding in patients with severe haemophilia A. Haemophilia 2009; 15:1210–1214.

    Article  CAS  Google Scholar 

  16. Centers for Disease Control and Prevention. Report on the Universal Data Collection Program. 2005; 7: 1–39. Available at hbd/documents/ UDC7(1).pdf.

  17. Pollmann H, Richter H, Ringkamp H, Jurgens H. When are children diagnosed as having severe haemophilia and when do they start to bleed? A 10-year single-centre PUP study. Eur J Pediatr 1999; 158(Suppl 3):S166–S166S170.

    Article  Google Scholar 

  18. Fischer K, van der Bom JG, Mauser-Bunschoten EP, Roosendaal G, Prejs R, de Kleijn P, et al. The effects of postponing prophylactic treatment on long-term outcome in patients with severe hemophilia. Blood 2002; 99:2337–2341.

    Article  CAS  Google Scholar 

  19. Knobe K, Berntorp E. Haemophilia and joint disease: pathophysiology, evaluation, and management. J Comorbidity 2011;1:51–59.

    Article  Google Scholar 

  20. Soucie JM, Cianfrini C, Janco RL, Kulkarni R, Hambleton J, Evatt B, et al. Joint range-of-motion limitations among young males with hemophilia: prevalence and risk factors. Blood 2004; 103:2467–2473.

    Article  CAS  Google Scholar 

  21. Raffini L, Manno C. Modern management of haemophilic arthropathy. Br J Haematol 2007; 136:777–787.

    Article  Google Scholar 

  22. Khawaji M, Astermark J, Von Mackensen S, Akesson K, Berntorp E. Bone density and health-related quality of life in adult patients with severe haemophilia. Haemophilia 2011; 17:304–311.

    Article  CAS  Google Scholar 

  23. Blanchette P, Rivard G, Israels S, Robinson S, Ali K, Walker I, et al. A survey of factor prophylaxis in the Canadian haemophilia A population. Haemophilia 2004; 10:679–683.

    Article  CAS  Google Scholar 

  24. Hassan TH, Badr MA, El-Gerby KM. Correlation between musculoskeletal function and radiological joint scores in haemophilia A adolescents. Haemophilia 2011; 17:920–925.

    Article  CAS  Google Scholar 

  25. Tlacuilo-Parra A, Villela-Rodriguez J, Garibaldi-Covarrubias R, Soto-Padilla J, Orozco-Alcala J. Functional independence score in hemophilia: a cross-sectional study assessment of Mexican children. Pediatr Blood Cancer 2010; 54:394–397.

    Article  Google Scholar 

  26. Valentino LA. Blood-induced joint disease: the pathophysiology of hemophilic arthropathy. J Thromb Haemost 2010; 8:1895–1902.

    Article  CAS  Google Scholar 

  27. Erlemann R, Pollmann H, Reiser M, Almeida P, Peters PE. Staging of hemophilic osteoarthropathy using the Pettersson score. Pathogenesis, early diagnosis, and prophylaxis for chronic hemophilic synovitis. Clin Orthop Relat Res 1997; 343:74–80.

    Google Scholar 

  28. Van Dijk K, Fischer K, van der Bom JG, Grobbee DE, van den Berg HM. Variability in clinical phenotype of severe haemophilia: the role of the first joint bleed. Haemophilia 2005; 11:438–443.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations


Corresponding author

Correspondence to Hayam M. Abdel Ghany MD.

Additional information

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

Rights and permissions

This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Abdel Ghany, H.M., Hassab, H.M.A. & El-Noueam, K.I. Hemophilic arthropathy: clinical, radiologic, and functional evaluation: a single-center experience in a limited resource country. Egypt Rheumatol Rehabil 43, 35–40 (2016).

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI:


  • Functional Independence Score in Hemophilia
  • hemophilic arthropathy
  • Pettersson scoring system