Soleimani Samarkhazan Hamed, Khaksari Mohammad Navid, Rahmati Ali, Esfahani Mahsa Loran, Solouki Amin, Aghaei Mojtaba
Student Research Committee, Department of Hematology and Blood Banking, School of Allied Medical Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Department of Hematology and Blood Banking, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
Thromb J. 2025 Apr 28;23(1):41. doi: 10.1186/s12959-025-00727-7.
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)是一种遗传性出血性疾病,其特征是血管性血友病因子(VWF)存在数量或质量缺陷。妊娠会显著影响止血功能,导致血液高凝状态。然而,由于妊娠相关的激素变化与VWF缺陷之间的相互作用,患有VWD的女性面临着独特的挑战。本综述深入探讨了VWD与妊娠之间的复杂关系。我们探究了妊娠期间发生的生理变化,包括激素波动、血液稀释以及血小板与VWF相互作用的改变。我们讨论了这些变化如何加剧患有VWD的女性的出血倾向,尤其是在分娩和产后期间。本综述强调了患有VWD的女性产后出血(PPH)风险增加以及严重孕产妇发病和死亡的可能性。我们研究了VWD的各种类型及其对妊娠结局的具体影响。此外,我们讨论了妊娠期间诊断和管理VWD的相关挑战,以及产前咨询和仔细监测的重要性。妊娠期间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,以及在必要时进行因子替代治疗。仔细规划分娩,包括选择分娩方式和干预时机,对于将出血并发症降至最低至关重要。通过了解妊娠期间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患有VWD的女性及其后代的结局。