Lee Christine A, Abdul-Kadir Rezan
Katharine Dormandy Haemophilia Center and Haemostasis Unit, Royal Free Hospital, London, United Kingdom.
Semin Hematol. 2005 Jan;42(1):42-8. doi: 10.1053/j.seminhematol.2004.11.002.
In 1926 von Willebrand described a bleeder family in Aland; this condition became known as von Willebrand disease (VWD). von Willebrand noted that "the trait seemed especially to be seen among the women." Today, the use of a pictorial bleeding assessment chart (PBAC) has enabled the prevalence of VWD to be established among women presenting with menorrhagia, as well as the documentation of this symptom in women with known VWD and the assessment of treatment response in menorrhagia. Treatments for menorrhagia include tranexamic acid, desmopressin (DDAVP) administered either intranasally or subcutaneously, the oral contraceptive pill, the "Mirena" coil (Schering Oy, Turku, Finland), and endometrial ablation. Von Willebrand factor (VWF) shows strong cyclical variation, with peak values occurring in the luteal phase. Although increased in pregnancy, levels of VWF decline postnatally and the incidence of both primary and secondary postpartum hemorrhage is high (20% to 25%). Baseline VWF levels less than 15 IU/dL are unlikely to reach greater than 50 IU/dL in the third trimester, and therefore prophylaxis with DDAVP or VWF-containing concentrate to cover delivery should be considered.
1926年,冯·威勒布兰德描述了奥兰群岛的一个出血者家族;这种病症后来被称为冯·威勒布兰德病(VWD)。冯·威勒布兰德指出,“这种特征似乎在女性中尤为常见。”如今,使用图片式出血评估图表(PBAC)已能够确定月经过多女性中VWD的患病率,记录已知患有VWD女性的这一症状,并评估月经过多的治疗反应。月经过多的治疗方法包括氨甲环酸、经鼻或皮下注射的去氨加压素(DDAVP)、口服避孕药、“曼月乐”环(芬兰图尔库的先灵公司)以及子宫内膜消融术。血管性血友病因子(VWF)呈现出强烈的周期性变化,在黄体期达到峰值。虽然孕期VWF水平会升高,但产后其水平会下降,原发性和继发性产后出血的发生率都很高(20%至25%)。妊娠晚期基线VWF水平低于15 IU/dL不太可能升至大于50 IU/dL,因此应考虑使用DDAVP或含VWF的浓缩物进行预防以应对分娩。