Open AccessCONGENITAL FACTOR V DEFICIENCY: CASE REPORTS
Author(s) -
Е. В. Яковлева,
Надежда Ивановна Коняшина,
Л. А. Горгидзе,
В. Л. Сурин,
О. С. Пшеничникова,
О. А. Полеводова,
М. В. Спирин,
Г М Галстян,
Н. И. Зозуля
Publication year - 2019
Publication title -
gematologiâ i transfuziologiâ
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.126
H-Index - 5
eISSN - 2411-3042
pISSN - 0234-5730
DOI - 10.35754/0234-5730-2019-64-4-489-503
Subject(s) - medicine , pediatrics , haemophilia , haemophilia a , disease , intensive care medicine
. 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.