P107 Prevalence of haemarthrosis and clinical impact on the musculoskeletal system in people with haemophilia in the United Kingdom: evaluation of UKHCDO and haemtrack patient reported data

Abstract

Introduction: Haemarthrosis (joint bleeds) is a significant feature of haemophilia. Despite the availability of coagulation factor concentrate prophylaxis in the UK, haemarthrosis is associated with worsening joint health and haemarthropathy, especially in people with severe haemophilia. Whilst annual joint bleed rates (AJBR) are commonly reported in haemophilia research, there is a lack of information on bleed rates and joint health in individual joints. Method(s): In 2018 haemarthrosis prevalence was identified in paediatric (<18 years) and adult (>=18 years) cases with severe haemophilia A and B registered with the UK Haemophilia Centres Doctors Organisation (UKHCDO) National Haemophilia Database (NHD). Fully itemised Haemophilia Joint Health Scores (HJHS) were obtained from NHD records in combination with AJBR from Haemtrack (HT) compliant patients. Result(s): 2238 cases were identified; 273 reported >=75% simultaneous HT compliance and electronic fully itemised HJHS data. The median (IQR) age of children was 10 (6-13) and adults 40 (29-50) years. The prevalence of joint bleeding in haemophilia A and B children was 33% and 47% respectively, and in adults 60% and 42% respectively. In children with haemophilia A (n = 80) the knee joint was the most common bleed site. In haemophilia A adults (n = 157) the ankle and elbow joints were most common. In haemophilia B children (n = 17) and adults (n = 19) the elbow was the most prevalent site. Total HJHS scores in children with haemophilia A and B were 0.00 (0.00; 0.00). Median total HJHS scores in adults with haemophilia A and B were 18.00 (6.00; 31.00) and 11.00 (5.00; 24.00) respectively. In adults with haemophilia A and haemophilia B, mean (3.80) and median (4.00) scores for the ankle joint were higher than for the knee (2.90; 1.00) and elbow (3.30; 1.00) joint. Discussion/Conclusion: Whilst there are limitations to this self-selected subset of cases with severe haemophilia, the ankle and elbow joints were reported to be the most prevalent sites of haemarthrosis in adults, and the knee in children. Despite prophylaxis, 30% and 60% of children and adults respectively reported joint bleeds over a 12 month period. HJHS of zero in children suggest changes do not present until later years. In adults deemed adherent with prophylaxis and HT reporting, there were worse HJHS scores despite a lower AJBR.