Pregnancy and delivery in women with von Willebrand disease.
Pregnancy and delivery in women with von Willebrand disease.
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DOI：
10.1111/ejh.13250
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发表时间：
2019-08
影响因子：
3.1
通讯作者：
James PD
中科院分区：
文献类型：
--
作者：
Castaman G;James PD
鉴于广泛的异质性的表型和潜在的病理生理机制与疾病，妊娠和分娩的血管性血友病（VWD）代表了一个重大的临床挑战。妊娠期间观察到的血管性血友病因子（VWF）和因子VIII（FVIII）（VWF携带的蛋白质）的变化模式可变，提示对VWD孕妇进行仔细评估，以计划分娩时最合适的治疗。然而，在妊娠期间也有可能需要紧急止血治疗以防止出血的情况（胎盘穿刺术、与胎盘剥离相关的阴道出血、突然流产）。因此，患有VWD的女性应该在对其类型，亚型和治疗进行充分表征后开始怀孕。VWF和FVIII基础水平>30 U/dL的VWD妇女通常在妊娠结束时使这些水平正常化，很少需要特异性抗出血预防。相反，那些基础水平<20 U/dL的人通常表现出较小的增加，需要特定的治疗。一些与清除率增加相关的突变的妇女可以用去氨加压素治疗，而那些对这种药物无反应或有禁忌症的妇女需要替代治疗。对于后者的妇女，怀孕期间阴道出血的风险可能会增加，需要使用VWF浓缩物进行预防。同样，在整个妊娠期间保持VWF活性降低的2型VWD女性需要使用FVIII/VWF浓缩物进行替代治疗。当替代治疗几天不继续时，可能会发生迟发性产后出血。氨甲环酸是有用的，在出院，以避免恶露过多。
Given the wide heterogeneity of phenotypes and of the underlying pathophysiological mechanisms associated with the disorder, pregnancy and delivery in von Willebrand disease (VWD) represent a significant clinical challenge. The variable pattern of changes observed during pregnancy of von Willebrand factor (VWF) and factor VIII (FVIII), the protein carried by VWF, prompts a careful evaluation of pregnant women with VWD to plan the most appropriate treatment at the time of parturition. However, there are also instances during pregnancy (amniocentesis, vaginal bleeding associated with placental detachment, sudden abortion) that may require urgent hemostatic treatment to prevent bleeding. Thus, women with VWD should start pregnancy after being well characterized as to their type, subtype, and treatments. Women with VWD who have VWF and FVIII basal levels >30 U/dL typically normalize these levels at the end of pregnancy and specific anti-hemorrhagic prophylaxis is seldom required. On the contrary, those with basal levels <20 U/dL usually show a lesser increase and specific treatment is required. Some women with mutations associated with increased clearance can be treated with desmopressin, while those unresponsive or with contra-indications to this agent need replacement therapy. For these latter women, the risk of vaginal bleeding during pregnancy may be increased and prophylaxis with VWF concentrates required. Similarly, women with type 2 VWD who maintain reduced VWF activity throughout pregnancy require replacement therapy with FVIII/VWF concentrates. Delayed post-partum bleeding may occur when replacement therapy is not continued for some days. Tranexamic acid is useful at discharge to avoid excessive lochia.
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