CONGENITAL FACTOR V DEFICIENCY: CASE REPORTS
E. V. Yakovleva,
N. I. Konyashina,
L. A. Gorgidze,
V. L. Surin,
O. S. Pshenichnikova,
O. A. Polevodova,
M. V. Spirin,
G. M. Galstyan,
N. I. Zozulya https://doi.org/10.35754/0234-5730-2019-64-4-489-503
Abstract
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.
About the Authors
E. V. Yakovleva
National Research Center for Hematology
Russian Federation
Russian Federation
Elena V. Yakovleva, Researcher, Cand. Sci. (Med.), Hematologist, Coagulopathy Department
tel.: +7 (495) 612-29-12
N. I. Konyashina
National Research Center for Hematology
Russian Federation
Nadezhda I. Konyashina, Cand. Sci. (Med.), Clinical Pathologist, Express Laboratory, Resuscitation and Intensive Care Unit
Russian Federation
Nadezhda I. Konyashina, Cand. Sci. (Med.), Clinical Pathologist, Express Laboratory, Resuscitation and Intensive Care Unit
L. A. Gorgidze
National Research Center for Hematology
Russian Federation
Lana A. Gorgidze, Cand. Sci. (Biol.), Senior Researcher in Clinical Pathology, Express Laboratory, Resuscitation and Intensive Care Unit
Russian Federation
Lana A. Gorgidze, Cand. Sci. (Biol.), Senior Researcher in Clinical Pathology, Express Laboratory, Resuscitation and Intensive Care Unit
V. L. Surin
National Research Center for Hematology
Russian Federation
Vadim L. Surin, Senior Researcher, Laboratory of genetic engineering
Russian Federation
Vadim L. Surin, Senior Researcher, Laboratory of genetic engineering
O. S. Pshenichnikova
National Research Center for Hematology
Russian Federation
Olesya S. Pshenichnikova, Senior Researcher, Laboratory of genetic engineering
Russian Federation
Olesya S. Pshenichnikova, Senior Researcher, Laboratory of genetic engineering
O. A. Polevodova
National Research Center for Hematology
Russian Federation
Olesya A. Polevodova, Intensivist, Resuscitation and Intensive Care Unit
Russian Federation
Olesya A. Polevodova, Intensivist, Resuscitation and Intensive Care Unit
M. V. Spirin
National Research Center for Hematology
Russian Federation
Russian Federation
Mikhail V. Spirin, Cand. Sci. (Med.), doctor, Resuscitation and Intensive Care Unit
tel.: +7 (926) 983-11-39
G. M. Galstyan
National Research Center for Hematology
Russian Federation
Russian Federation
Gennady M. Galstyan, Dr. Sci. (Med.), Head of the Resuscitation and Intensive Care Unit
tel.: +7 (495) 612-48-59
N. I. Zozulya
National Research Center for Hematology
Russian Federation
Nadezhda I. Zozulya, Dr. Sci. (Med.), Hematologist, Head of the Scientific and consulting department of coagulopathies
Russian Federation
Nadezhda I. Zozulya, Dr. Sci. (Med.), Hematologist, Head of the Scientific and consulting department of coagulopathies
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For citations:
Yakovleva E.V., Konyashina N.I., Gorgidze L.A., Surin V.L., Pshenichnikova O.S., Polevodova O.A., Spirin M.V., Galstyan G.M., Zozulya N.I. CONGENITAL FACTOR V DEFICIENCY: CASE REPORTS. Russian journal of hematology and transfusiology. 2019;64(4):489–503. (In Russ.) https://doi.org/10.35754/0234-5730-2019-64-4-489-503
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