Department of Medicine, Medical Clinic 2, Institute of Transfusion Medicine, University Hospital, Goethe University, Frankfurt, Germany.
Hamostaseologie. 2022 Oct;42(5):330-336. doi: 10.1055/a-1891-9976. Epub 2022 Nov 2.
Von Willebrand disease (VWD) is the most common inherited bleeding disorder, which results from a deficiency or dysfunction of von Willebrand factor (VWF). The major symptoms of patients affected by VWD include mucocutaneous and gastrointestinal bleeding, easy bruising, and prolonged provoked bleeding due to injury or surgery. Although women and men are equally likely to be affected by VWD, women continue to be disproportionately affected by the bleeding challenges. Women with VWD suffer from sex-specific symptoms, such as menorrhagia, and are at higher risk of reproductive problems and recurrent miscarriage. Furthermore, pregnant women with VWD are more likely at higher risk of suffering from primary and secondary peripartal hemorrhage and anemia and the need for transfusions. Despite being affected by gynecologic and obstetrical bleeding, women face multiple barriers in obtaining an accurate diagnosis. This constitutes a problem that needs to be addressed, and early appropriate medical care should be ensured. There are several effective treatment options for women with VWD that can significantly improve their quality of life, including desmopressin, VWF concentrates, hormonal therapy, and antifibrinolytic therapy. During pregnancy, the monitoring of VWF activity levels is essential. The peripartal management depends on the type of VWD and on the measured levels of VWF levels and activity prior to delivery.
血管性血友病（von Willebrand disease，VWD）是最常见的遗传性出血性疾病，由血管性血友病因子（von Willebrand factor，VWF）缺乏或功能异常引起。VWD 患者的主要症状包括黏膜皮肤和胃肠道出血、容易瘀伤以及因创伤或手术引起的延长性诱发性出血。尽管男性和女性同样容易受到 VWD 的影响，但女性仍然因出血问题受到不成比例的影响。患有 VWD 的女性会出现与性别相关的症状，如月经过多，并且生殖问题和反复流产的风险更高。此外，患有 VWD 的孕妇更容易出现原发性和继发性围产期出血和贫血，并需要输血。尽管患有妇科和产科出血，但女性在获得准确诊断方面面临多种障碍。这是一个需要解决的问题，应确保尽早进行适当的医疗护理。VWD 女性有多种有效的治疗选择，可以显著提高生活质量，包括去氨加压素、VWF 浓缩物、激素治疗和抗纤维蛋白溶解治疗。在怀孕期间，监测 VWF 活性水平至关重要。围产期管理取决于 VWD 的类型以及分娩前 VWF 水平和活性的测量值。
