Inherited disorders of hemostasis in the pathogenesis of obstetric pathology

Relevance. Complications of pregnancy and childbirth are a pressing issue in obstetric practice. The study of cases of repeated fetal loss, stillbirth, intrauterine growth retardation and preeclampsia leads to the discovery of new aspects of this pathology. Of particular interest is Upshaw - Shulman syndrome (USS), a rare congenital form of thrombotic thrombocytopenic purpura (TTP) caused by mutations in the ADAMTS13 gene. This gene encodes a metalloproteinase capable of cleaving von Willebrand factor (vWF), which is important for blood clotting processes. The aim of the study is to identify the links in the pathogenesis of clinical manifestations and highlight current trends in the identification of patients suffering from reproductive insufficiency in association with Upshaw - Shulman syndrome. The disease caused by the delay of ultrahigh molecular weight vWF multimers in the bloodstream leads to vascular microthrombosis. Recent studies involving pregnant women have shown that the heterogeneity of manifestations complicates the diagnosis of this pathology. The disease occurs suddenly and is associated with an increased risk of death of both mother and fetus. As part of the study, the following tasks were set: to analyze the relevance of the diagnosis of the ADAMTS13 genetic defect in Upshaw - Shulman syndrome, to determine the practicality and effectiveness of technologies for the treatment of emerging obstetric complications. A review of modern Russian and foreign literature, mainly in English, devoted to the methods of diagnosis and treatment of congenital thrombocytopenic purpura, as well as the prevention of complications of pregnancy and childbirth, was conducted. The latest publications in specialized medical journals were studied. This review highlights the main mechanisms of development and progression of the syndrome, current directions of management of women with Upshaw - Shulman syndrome and methods of therapy for associated reproductive failure, as well as modern groups of pharmacological drugs of choice. Conclusion . It was found that in 30 % of cases, the actual first-line treatment remains ineffective. However, the correct treatment strategy for patients with an increased risk of miscarriage can significantly reduce the incidence of maternal and perinatal morbidity and mortality caused by genetic defects in the hemostasis system. Pregnancy with Upshaw - Shulman syndrome is becoming one of the main vectors of research in the field of obstetrics, so it is important to conduct further high-quality associative research to develop innovative therapeutic methods and opportunities in the future.