Kadir Rezan A, Chi Claudia
Department of Obstetrics Gynaecology, Royal Free Hospital, London, UK.
Semin Thromb Hemost. 2006 Sep;32(6):605-15. doi: 10.1055/s-2006-949665.
von Willebrand disease (vWD) is the commonest bleeding disorder in women. More than 70% of women with vWD suffer from menorrhagia and half of them suffer from dysmenorrhea. There is also the risk of hemorrhage with ovulation and mid-cycle pain. These have a significant effect on all aspects of quality of life. vWD also can be the underlying cause of menorrhagia in a small but significant proportion of women. There are still several unanswered issues in the diagnosis and management of menorrhagia in these women. There is no consensus whether testing for vWD should be part of the routine investigations in menorrhagia. Diagnosis of vWD is difficult. There are intraindividual variations in von Willebrand factor and factor VIII levels influenced by age, race, and blood group. This is further complicated in women because of the fluctuation of these factor levels during the menstrual cycle and possibly with hormonal therapy. The diagnosis of menorrhagia is also difficult due to the lack of a simple objective tool for the assessment of menstrual blood loss. In vWD, the treatment of menorrhagia is usually medical, but there is lack of prospective data on the efficacy of commonly used medical therapies in these women. The levonorgestrel intrauterine system, Mirena, is effective and should be considered prior to surgical management. Surgical interventions may be required in patients unresponsive to medical treatments. These procedures can be complicated by hemorrhage in these women. A multidisciplinary approach in the management of these women is essential in ensuring an optimal outcome. Multicenter clinical trials are required to answer the controversial issues in the management of women with vWD.
血管性血友病（vWD）是女性中最常见的出血性疾病。超过70%的vWD女性患者患有月经过多，其中一半患有痛经。排卵时也有出血风险以及月经周期中期疼痛。这些对生活质量的各个方面都有显著影响。vWD在一小部分但比例可观的女性中也可能是月经过多的潜在原因。在这些女性月经过多的诊断和管理方面仍有几个未解决的问题。对于vWD检测是否应作为月经过多常规检查的一部分，尚无共识。vWD的诊断很困难。血管性血友病因子和因子VIII水平存在个体内差异，受年龄、种族和血型影响。在女性中情况更复杂，因为这些因子水平在月经周期中会波动，并且可能受激素治疗影响。由于缺乏评估月经量的简单客观工具，月经过多的诊断也很困难。在vWD中，月经过多的治疗通常是药物治疗，但缺乏关于这些女性常用药物治疗疗效的前瞻性数据。左炔诺孕酮宫内节育系统（曼月乐）有效，在手术治疗前应予以考虑。对药物治疗无反应的患者可能需要手术干预。这些手术在这些女性中可能会因出血而复杂化。对这些女性进行多学科管理对于确保最佳结果至关重要。需要进行多中心临床试验来回答vWD女性管理中的争议性问题。
