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首页> 美国卫生研究院文献>Mediterranean Journal of Hematology and Infectious Diseases >Changes of von Willebrand Factor during Pregnancy in Women with and without von Willebrand Disease
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Changes of von Willebrand Factor during Pregnancy in Women with and without von Willebrand Disease

机译：von Willebrand病和无von Willebrand病的女性在怀孕期间von Willebrand因子的变化

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Delivery in von Willebrand disease (VWD) represents a significant hemostatic challenge because of the variable pattern of changes observed during pregnancy of von Willebrand factor (VWF) and factor VIII (FVIII), the protein carried by VWF. Since a wide heterogeneity of phenotypes and of the underlying pathophysiological mechanisms is associated with this disorder, a prompt and careful evaluation of pregnant women with VWD is requested in order to plan the most appropriate treatment at time of parturition. VWF and FVIII increase significantly during pregnancy in normal women, already within the first trimester, reaching levels by far >100 U/dL by the time of parturition. Women with VWD, levels at baseline of VWF and FVIII >30 U/dL have us a high likelihood to achieve normal levels at the end of pregnancy; thus specific anti-hemorrhagic prophylaxis is seldom required. Women with basal level <20 U/dL usually have a poor increase since most of these women carry mutations associated with increased VWF clearance or are compound heterozygous for different VWF mutations; that prevent the achievement of satisfactory hemostatic levels. While women with mutations associated with increased clearance show a full, albeit transitory correction of their hemostatic deficiency after desmopressin administration, compound heterozygous need replacement therapy because they do not respond well to this agent. Patients with abnormal VWF:RCo/VWF:Ag ratio at baseline (e.g. <0.6), typically associated with type 2 VWD, maintain the abnormality throughout pregnancy and VWF:RCo usually does not attain safe levels ≥50 U/dL. These women require replacement therapy with VWF-FVIII concentrates. Delayed post-partum bleeding may occur when replacement therapy is not continued for some days. Tranexamic acid may be useful at discharge to avoid excessive lochia.
机译：von Willebrand病（VWD）的递送代表着重大的止血挑战，因为在怀孕期间观察到von Willebrand因子（VWF）和VIII因子（FVIII）（由VWF携带的蛋白质）的变化模式。由于表型和潜在的病理生理机制的广泛异质性与该疾病有关，因此要求对VWD孕妇进行迅速而仔细的评估，以计划分娩时最合适的治疗。正常妇女在怀孕期间的VWF和FVIII显着增加，早在怀孕的三个月之内，到分娩时已达到> 100 U / dL的水平。 VWD，VWF基线水平和FVIII> 30 U / dL的女性在妊娠末期极有可能达到正常水平；因此，几乎不需要特殊的抗出血预防措施。基础水平<20 U / dL的女性通常增长不佳，因为这些女性中的大多数携带与增加的VWF清除率相关的突变，或者是针对不同VWF突变的复合杂合体。妨碍达到令人满意的止血水平。尽管在应用去氨加压素后出现与清除率增加相关的突变的妇女显示出其止血功能的暂时性完全纠正，但复合杂合子仍需要替代疗法，因为她们对此药的反应不佳。基线时VWF：RCo / VWF：Ag比率异常（例如<0.6）的患者（通常与2型VWD相关）在整个妊娠期间均保持异常，并且VWF：RCo通常不能达到≥50 U / dL的安全水平。这些妇女需要使用VWF-FVIII浓缩液替代治疗。如果替代疗法持续几天没有发生，可能会延迟产后出血。氨甲环酸在排出时可能有用，以避免恶露过多。

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