Scharrer I
Haemophilia Center, University Hospital Frankfurt am Main, Theodor-Stern-Kai 7, 60590 Frankfurt am Main, Germany.
Hamostaseologie. 2004 Feb;24(1):44-9. doi: 10.1267/hamo04010044.
The clinical presentation of VWD shows sex-related differences of symptoms. In women the most typical symptoms are menorrhagia, bleeding during and after delivery or abortion and bleeding in connection with caesarean section or gynaecological surgery. Menorrhagia is one of the most common symptoms presented to gynaecologists. In 7-20% of menorrhagia the underlying cause is VWD, in our cohort of 185 women with menorrhagia the prevalence of VWD was even 32%. On the other hand in women with VWD menorrhagia with onset at the menarche can be found in 60-93%, influencing substantially their morbidity and quality of life. During pregnancy women with mild VWD experience a decrease of bleeding tendency due to an increase of endogenous VWF. But as the VWF concentration drops rapidly after delivery, the post-partum period is often associated with significant bleeding complications. In severe forms of VWD the bleeding risk is high during delivery and postpartum period. Laboratory monitoring and therapeutical measures should be continued for 8-10 days after delivery. During menopause the clinical situation improves for most of the women with mild or moderate VWD.
血管性血友病（VWD）的临床表现存在性别相关的症状差异。在女性中，最典型的症状是月经过多、分娩或流产期间及之后出血，以及与剖宫产或妇科手术相关的出血。月经过多是妇科医生接诊的最常见症状之一。在7%至20%的月经过多病例中，潜在病因是血管性血友病，在我们185例月经过多女性的队列中，血管性血友病的患病率甚至达到32%。另一方面，在血管性血友病女性中，60%至93%的患者初潮时即出现月经过多，这对她们的发病率和生活质量有很大影响。在孕期，轻度血管性血友病女性由于内源性血管性血友病因子（VWF）增加，出血倾向会降低。但由于产后VWF浓度迅速下降，产后时期常伴有严重出血并发症。在严重的血管性血友病病例中，分娩期间及产后出血风险很高。分娩后应持续进行实验室监测和治疗措施8至10天。在绝经期间，大多数轻度或中度血管性血友病女性的临床情况会有所改善。
