National Haemophilia Centre, University Hospital Centre Zagreb, Rebro, Zagreb, Croatia.
Haemophilia. 2011 Jan;17(1):95-102. doi: 10.1111/j.1365-2516.2010.02265.x.
Rapid control of bleeding is the key to reducing bleeding complications and thereby preserving joint and musculoskeletal function in haemophilia patients with inhibitors. However, this requires early diagnosis following the onset of bleeding and strategies for rapid treatment in an outpatient setting. Overarching themes on the need for speed in managing bleeds in haemophilia patients were examined by a panel of clinicians experienced in managing inhibitor patients and joint disease during the Third Zürich Haemophilia Forum on 8 May 2009. This report summarizes the opinions of the panel on how to achieve rapid bleeding control in inhibitor patients and areas that were identified by the panel for future research or as needing new consensus guidelines. The consensus was that home treatment should be established for haemophilia patients with inhibitors, as it is associated with a faster time to treatment, as well as improvements in the quality of life of patients and their carers. In addition, as improved haemostatic control now allows inhibitor patients to participate in a wider range of physical activities, specific guidelines are required on which types of sport and work are appropriate. It was agreed that clear, systematic approaches are needed for early diagnosis of joint and muscle bleeds in inhibitor patients, which could facilitate rapid treatment. There may be opportunities for exploiting new diagnostic techniques from osteoarthritis to enable earlier diagnosis of haemophilic arthropathy. Overall, it was concluded that greater emphasis should be placed on education and patients' psychological needs, to enable inhibitor patients to cope up more effectively with their disease.
快速控制出血是减少出血并发症的关键,从而保留血友病伴抑制剂患者的关节和肌肉骨骼功能。然而,这需要在出血发生后尽早诊断,并在门诊环境中制定快速治疗策略。在 2009 年 5 月 8 日的第三届苏黎世血友病论坛上,一组在管理抑制剂患者和关节疾病方面经验丰富的临床医生探讨了在管理血友病患者出血时需要快速的主题。本报告总结了专家组对如何在抑制剂患者中实现快速出血控制的意见,以及专家组确定的未来研究领域或需要新共识指南的领域。专家组一致认为,应该为血友病伴抑制剂患者建立家庭治疗,因为这与更快的治疗时间以及患者及其护理人员生活质量的提高有关。此外,由于更好的止血控制现在允许抑制剂患者参与更广泛的体育活动,因此需要特定的指南来确定哪些类型的运动和工作是合适的。专家组一致认为,需要明确、系统的方法来早期诊断抑制剂患者的关节和肌肉出血,这可以促进快速治疗。从骨关节炎中利用新的诊断技术可能有机会更早地诊断血友病性关节炎。总体而言,结论是应该更加重视教育和患者的心理需求,以使抑制剂患者能够更有效地应对他们的疾病。