Obstetric and perinatal risks in patients with von Willebrand disease

This lecture is devoted to a common inherited hemorrhagic disorder, von Willebrand disease (vWD). The article presents the history of the discovery of vWD, the structure and function of von Willebrand factor (vWF), the classification of vWD and its main clinical manifestations. The central topic of the lecture is vWD in pregnant women. It is indicated that to date there are no proven guidelines for the treatment of women with vWD during pregnancy, childbirth, and in the postpartum period. Due to the risk of bleeding, the management of pregnant women with vWD, especially those with its severe form, should be performed by a multidisciplinary team including a hematologist, obstetrician-gynecologist, anesthesiologist, and neonatologist. In patients with type 1 vWD, pregnancy is often uncomplicated, but there is always the risk of bleeding. Type 2 vWD (qualitative variants) requires continuous monitoring. In type 3 vWD, the most clinically severe variant, continuous monitoring of patients is also necessary, with initiation of replacement therapy (factor VIII and vWF) until the end of pregnancy. Conclusion. Von Willebrand disease is a heterogeneous disorder with a highly variable clinical presentation. It carries a high risk of prenatal and postpartum hemorrhage, therefore vWD patients are more likely to require blood transfusion. Since the sample of patients with vWD is quite small, further observations and studies are needed to obtain more reliable results. Key words: von Willebrand disease, von Willebrand factor, classification, clinical picture, diagnosis during pregnancy, management of pregnancy and childbirth