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Introduction: Persons with inherited bleeding disorders are at a substantial risk of bleeding following dental procedures., Aim: To compare the outcomes and use of haemostatic treatment pre- and post-implementation of a standardized protocol for dental procedures at a Hemophilia Treatment Centre., Methods: We conducted a retrospective cohort study of outpatient and inpatient dental procedures and maxillofacial surgeries sustained by people with bleeding disorders treated at a comprehensive Hemophilia Treatment Centre (2013-2020), comparing patients' outcomes before and after the introduction of the protocol in 2018. The protocol, built using a multidisciplinary approach, suggested haemostatic treatment based on the invasiveness of the dental procedure and the proposed anaesthesia. Our primary outcome was the rate of procedural bleeding leading to medical or dental reintervention within 10 days. Secondary outcomes included the use of systemic haemostatic treatment and treatment-related adverse effects., Results: Overall, 137 dental procedures in 95 patients (median age: 29 years; 78% males; 74% haemophilia, 14% von Willebrand disease, 12% other disorders) were included. Seventeen procedural bleedings were reported (12.4%). Procedural bleeding occurred in 14.8% and 8.9% of patients in the control and intervention groups (p = .304). No major bleeding occurred. Tranexamic acid was used more consistently after protocol implementation (72.8% vs. 89.3%, p = .019), while factor concentrates use decreased (65.4% vs. 44.6%, p = .016), and desmopressin use remained constant (46.4% vs. 32.1%, p = .100). No treatment-related adverse effects were reported., Conclusion: The use of a standardized protocol increased the use of tranexamic acid, with a nonstatistically significant reduction in procedural bleeding rate., (© 2022 John Wiley & Sons Ltd.)

Objective: The primary objective of this study was to assess whether there are different patterns (classes) of joint health in young boys with severe haemophilia A (SHA) prescribed primary tailored prophylaxis. We also assessed whether age at first index joint bleed, blood group, FVIII gene abnormality variant, factor VIII trough level, first-year bleeding rate and adherence to the prescribed prophylaxis regimen significantly predicted joint damage trajectory, and thus class membership., Methods: Using data collected prospectively as part of the Canadian Hemophilia Primary Prophylaxis Study (CHPS), we implemented a latent class growth mixture model technique to determine how many joint damage classes existed within the cohort. We used a multinomial logistic regression to predict the odds of class membership based on the above predictors. We fitted a survival model to assess whether there were differences in the rate of dose escalation across the groups., Results: We identified three distinct classes of trajectory: persistently low, moderately increasing and rapidly increasing joint scores. By multinomial regression, we found that only age at first index joint bleed predicted rapidly increasing joint scores. The rapidly increasing joint score class group moved through dose escalation significantly faster than the other two groups., Conclusions: Using tailored prophylaxis, boys with SHA follow one of three joint health trajectories. By using knowledge of disease trajectories, clinicians may be able to adjust treatment according to a subject's predicted long-term joint health and institute cost-effective programmes of prophylaxis targeted at the individual subject level., (© 2021 John Wiley & Sons Ltd.)

Introduction: Acquired haemophilia A (AHA) is a rare autoimmune bleeding disorder caused by neutralizing antibodies against factor VIII (FVIII). Despite significant initial morbidity and mortality, most patients achieve remission with immunosuppressive therapy., Aim: Long-term follow-up data from the Quebec Reference Centre for Inhibitors (QRCI) were analysed to identify factors predictive of AHA relapse and the influence of relapse on survival., Methods: Criteria used to define AHA were levels of FVIII <0.3 IU/mL and FVIII inhibitor titres ≥0.6 Bethesda Units (BU). Complete remission was defined as FVIII >0.5 IU/mL and/or FVIII inhibitor titres <0.6 BU while not on immunosuppression., Results: Between 2000 and 2012, 111 subjects met the inclusion criteria and were followed for a median of 25.6 months. Ninety per cent of them reached remission on immunosuppression in a median time of 45 days. Fourteen patients presented one or more relapses in a median time of 13.4 months. Most relapse episodes were successfully treated. Associated lymphoproliferative syndromes (LPS) were predictive of relapse, whereas FVIII activity and inhibitor titres at initial diagnosis or immunosuppressive regimens were not. The overall survival (OS) was the same, with or without relapse., Conclusion: Among the recognized potential risk factors for relapse, only LPS was statistically significant. The long-term follow-up of our patients also showed that late or multiple relapses may occur, but that relapse is not associated with a worse OS. Thus, long-term follow-up is important for optimal management of AHA., (© 2019 John Wiley & Sons Ltd.)