Von Willebrand disease (VWD) and pregnancy: a comprehensive overview.

IF 2.6 4区 医学 Q2 HEMATOLOGY
Hamed Soleimani Samarkhazan, Mohammad Navid Khaksari, Ali Rahmati, Mahsa Loran Esfahani, Amin Solouki, Mojtaba Aghaei
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Abstract

Von Willebrand disease (VWD) is a hereditary bleeding disorder characterized by a quantitative or qualitative deficiency of von Willebrand factor (VWF). Pregnancy significantly impacts hemostasis, leading to a hypercoagulable state. However, women with VWD experience unique challenges due to the interplay between pregnancy-related hormonal changes and VWF deficiencies. This review delves into the intricate relationship between VWD and pregnancy. We explored the physiological changes that occur during pregnancy, including hormonal fluctuations, hemodilution, and alterations in platelet-VWF interactions. We discuss how these changes can exacerbate bleeding tendencies in women with VWD, particularly during childbirth and the postpartum period. This review highlights the increased risk of postpartum hemorrhage (PPH) in women with VWD and the potential for severe maternal morbidity and mortality. We examine the various types of VWD and their specific implications for pregnancy outcomes. Additionally, we discuss the challenges associated with diagnosing and managing VWD during pregnancy, as well as the importance of prenatal counseling and careful monitoring. The management of VWD during pregnancy involves a multidisciplinary approach, including the use of prophylactic treatments, such as desmopressin and tranexamic acid, as well as factor replacement therapy when necessary. Careful planning of delivery, including the choice of delivery mode and the timing of interventions, is essential to minimize bleeding complications. By understanding the complexities of VWD during pregnancy and implementing appropriate management strategies, healthcare providers can significantly improve the outcomes for women with VWD and their offspring.

血管性血友病(VWD)与妊娠:全面概述。
血管性血友病(VWD)是一种遗传性出血性疾病,以血管性血友病因子(VWF)的定量或定性缺乏为特征。妊娠显著影响止血,导致高凝状态。然而,由于妊娠相关激素变化和VWF缺乏之间的相互作用,患有VWD的妇女面临着独特的挑战。本文将深入探讨VWD与妊娠之间的复杂关系。我们探讨了怀孕期间发生的生理变化,包括激素波动、血液稀释和血小板- vwf相互作用的改变。我们讨论了这些变化如何加剧VWD妇女的出血倾向,特别是在分娩和产后期间。这篇综述强调了VWD妇女产后出血(PPH)的风险增加以及严重的孕产妇发病率和死亡率的可能性。我们研究了各种类型的VWD及其对妊娠结局的具体影响。此外,我们还讨论了与妊娠期VWD诊断和管理相关的挑战,以及产前咨询和仔细监测的重要性。妊娠期VWD的管理涉及多学科方法,包括使用预防性治疗,如去氨加压素和氨甲环酸,以及必要时的因子替代治疗。精心规划分娩,包括选择分娩方式和干预措施的时机,对于尽量减少出血并发症至关重要。通过了解妊娠期VWD的复杂性并实施适当的管理策略,医疗保健提供者可以显著改善VWD妇女及其后代的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Thrombosis Journal
Thrombosis Journal Medicine-Hematology
CiteScore
3.80
自引率
3.20%
发文量
69
审稿时长
16 weeks
期刊介绍: Thrombosis Journal is an open-access journal that publishes original articles on aspects of clinical and basic research, new methodology, case reports and reviews in the areas of thrombosis. Topics of particular interest include the diagnosis of arterial and venous thrombosis, new antithrombotic treatments, new developments in the understanding, diagnosis and treatments of atherosclerotic vessel disease, relations between haemostasis and vascular disease, hypertension, diabetes, immunology and obesity.
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