Prevention and treatment of musculoskeletal disease in the haemophilia population: role of prophylaxis and synovectomy
- 1 July 2006
- journal article
- review article
- Published by Wiley in Haemophilia
- Vol. 12 (s3), 159-168
- https://doi.org/10.1111/j.1365-2516.2006.01281.x
Abstract
Prophylaxis is defined as primary (started before the onset of joint damage) or secondary (started after the onset of joint damage). The aim of primary prophylaxis is to prevent recurrent bleeding into joints and the development of chronic arthropathy in later life. When started early, and at most after two joint bleeds, the result is predictably excellent if there is compliance with the primary prophylaxis regimen. In order to decrease the need for central venous access devices to assure reliable venous access, a number of centres start primary prophylaxis with once weekly infusions with dose-escalation based on frequency of joint bleeding. A major unanswered question is whether primary prophylaxis can be safely discontinued in adolescents/young adults and if so, when. A promising predictor for the milder bleeding phenotype in persons with severe haemophilia is a later onset of joint bleeding. Once joint damage has occurred as a result of recurrent bleeding, secondary prophylaxis can only retard, but not prevent, ongoing joint damage. Other strategies to decrease recurrent bleeding from target joints include surgical synovectomy (ideally performed using an arthroscopic technique), radionuclide synovectomy and chemical synovectomy. These interventions have very good outcomes when performed by an experienced team. Given the very high cost of factor concentrates required for programmes of prophylaxis prospective studies that document benefits to the child and family, e.g. quality of life are to be encouraged.Keywords
This publication has 53 references indexed in Scilit:
- Are randomized clinical trials the only truth? Not alwaysJournal of Thrombosis and Haemostasis, 2006
- Variability in clinical phenotype of severe haemophilia: the role of the first joint bleedHaemophilia, 2005
- Divide and conquer: stomping leukemia cells by stimulating them to growBlood, 2005
- Prophylactic recombinant factor VIIa in haemophilia patients with inhibitorsHaemophilia, 2005
- International ForumVox Sanguinis, 2005
- Optimizing factor prophylaxis for the haemophilia population: where do we stand?Haemophilia, 2004
- Consensus recommendations for use of central venous access devices in haemophiliaHaemophilia, 2004
- Erratum for vol. 103, p. 2908Blood, 2004
- Haemophilia prophylaxis in young patients–a long‐term follow‐upJournal of Internal Medicine, 1997
- A longitudinal study of orthopaedic outcomes for severe factor‐VIII‐deficient haemophiliacsJournal of Internal Medicine, 1994