НАСЛЕДСТВЕННЫЙ ДЕФИЦИТ ФАКТОРА СВЕРТЫВАНИЯ КРОВИ V: КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ

Introduction . Haemophilia and von Willebrand disease constitute the most common hereditary coagulopathies. However, such rare hereditary coagulopathies as congenital factor V defi ciency can mistakingly be referred to these diseases. Aim . To describe the clinical manifestations and treatment of congenital factor V defi ciency. General findings . The article presents a literature review, as well as three case studies of patients with congenital factor V deficiency. Given that the choice of haemostatic therapy depends on accurate diagnosis, issues associated with the differential diagnosis of hereditary coagulopathies are considered as well. Patients with congenital factor V deficiency require continuous monitoring by a haematologist in order to control spontaneous or induced haemorrhagic syndrome, as well as to plan haemostatic therapy in case of surgical procedures, pregnancy or childbirth. Conflict of interest: the authors declare no conflict of interest. Financial disclosure : the study had no sponsorship.