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Guideline

Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia: A United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO) guideline.

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Last updated:19th Jun 2018

Although the exact pathogenesis of haemophilic arthropathy has not been elucidated, several in vitro animal and human experiments have demonstrated bloodinduced changes in all the ‘components’ of large synovial joints including the synovium itself, cartilage and the subchondral bone. The two major changes in the synovium are hypertrophy and hypervascularity. The key stimulant for these changes is iron released into the synovial fluid, which is both pro-inflammatory and proangiogenic [2–4]. The consequence of neovascularisation in the synovium is predisposition to more bleeding since these new vessels are friable. This leads to a vicious circle of bleeding, iron accumulation, synovial hypertrophy and hypervascularization leading to further bleeding and ultimately progressive joint damage. Interventions aimed at preventing or breaking this cycle are key strategies for the preservation of joint function in people with haemophilia. Bleeding is particularly problematic in the diarthrodial-hinged joints such as the knee, elbow and
ankle. It is therefore important that acute bleeds in patients on either ‘on demand’ or ‘prophylaxis’ regimens are treated optimally with the aim of minimizing the synovial proliferation secondary to bleeding which is central to the pathogenesis of arthropathy.

A recent survey has shown significant variation in practice in the UK [5], which suggests that there is a need for guidelines as a framework for best practice. Evidence-based guidelines were developed summarizing best practice for the assessment and management of acute joint bleeds and chronic synovitis in persons with haemophilia. This guideline does not include surgical procedures such as surgical synovectomy, arthrodesis and arthroplasty.

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