bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2019-64-4-489-503bloodjour-169Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCASE REPORTSНАСЛЕДСТВЕННЫЙ ДЕФИЦИТ ФАКТОРА СВЕРТЫВАНИЯ КРОВИ V: КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCONGENITAL FACTOR V DEFICIENCY: CASE REPORTShttps://orcid.org/0000-0002-6991-7437ЯковлеваЕ. В.YakovlevaE. V.

Яковлева Елена Владимировна, научный сотрудник, кандидат медицинских наук, врач-гематолог отдела коагулопатий

тел.: +7 (495) 612-29-12

Elena V. Yakovleva, Researcher, Cand. Sci. (Med.), Hematologist, Coagulopathy Department 

tel.: +7 (495) 612-29-12

hemophilia2012@mail.ruКоняшинаН. И.KonyashinaN. I.

Коняшина Надежда Ивановна, кандидат медицинских наук, врач клинической лабораторной диагностики экспресс-лаборатории отделения реанимации и интенсивной терапии 

тел.: +7 (910) 477-58-75

Nadezhda I. Konyashina, Cand. Sci. (Med.), Clinical Pathologist, Express Laboratory, Resuscitation and Intensive Care Unit

konyashinan@mail.ruГоргидзеЛ. А.GorgidzeL. A.

Горгидзе Лана Анзоровна, кандидат биологических наук, старший научный сотрудник клинической лабораторной диагностики экспресс-лаборатории отделения реанимации и интенсивной терапии 

тел.: +7 (985) 021-70-21

Lana A. Gorgidze, Cand. Sci. (Biol.), Senior Researcher in Clinical Pathology, Express Laboratory, Resuscitation and Intensive Care Unit

lana380@mail.ruhttps://orcid.org/0000-0002-1890-4492СуринВ. Л.SurinV. L.

Сурин Вадим Леонидович, старший научный сотрудник лаборатории генной инженерии

Vadim L. Surin, Senior Researcher, Laboratory of genetic engineering

vadsurin@mail.ruhttps://orcid.org/0000-0001-5752-8146ПшеничниковаО. С.PshenichnikovaO. S.

Пшеничникова Олеся Сергеевна, старший научный сотрудник лаборатории генной инженерии

Olesya S. Pshenichnikova, Senior Researcher, Laboratory of genetic engineering

pshenichnikovaolesya@gmail.comhttps://orcid.org/0000-0002-7783-5861ПолеводоваО. А.PolevodovaO. A.

Полеводова Олеся Алексеевна, врач — анестезиолог-реаниматолог отделения реанимации и интенсивной терапии 

тел.: +7 (495) 612-48-59

Olesya A. Polevodova, Intensivist, Resuscitation and Intensive Care Unit

polevodova_md@mail.ruhttps://orcid.org/0000-0001-7048-060XСпиринМ. В.SpirinM. V.

Спирин Михаил Васильевич, кандидат медицинских наук, врач отделения реанимации и интенсивной терапии 

тел.: +7 (926) 983-11-39

Mikhail V. Spirin, Cand. Sci. (Med.), doctor, Resuscitation and Intensive Care Unit

tel.: +7 (926) 983-11-39

mvspirin@gmail.comhttps://orcid.org/0000-0001-8818-8949ГалстянГ. М.GalstyanG. M.

Галстян Геннадий Мартинович, доктор медицинских наук, заведующий отделением реанимации и интенсивной терапии 

тел.: +7 (495) 612-48-59

Gennady M. Galstyan, Dr. Sci. (Med.), Head of the Resuscitation and Intensive Care Unit

tel.: +7 (495) 612-48-59

gengalst@gmail.comhttps://orcid.org/0000-0001-7074-0926ЗозуляН. И.ZozulyaN. I.

Зозуля Надежда Ивановна, доктор медицинских наук, врач-гематолог, заведующая научно-консультативным отделом коагулопатий

Nadezhda I. Zozulya, Dr. Sci. (Med.), Hematologist, Head of the Scientific and consulting department of coagulopathies

zozulya.n@blood.ruФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения РоссииРоссияNational Research Center for HematologyRussian Federation201912122019644489–503Copyright © Яковлева Е.В., Коняшина Н.И., Горгидзе Л.А., Сурин В.Л., Пшеничникова О.С., Полеводова О.А., Спирин М.В., Галстян Г.М., Зозуля Н.И., 20192019Яковлева Е.В., Коняшина Н.И., Горгидзе Л.А., Сурин В.Л., Пшеничникова О.С., Полеводова О.А., Спирин М.В., Галстян Г.М., Зозуля Н.И.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.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.htjournal.ru/jour/article/view/169Введение

Введение. Наиболее распространенными наследственными коагулопатиями являются гемофилия и болезнь Виллебранда. Часто под маской этих заболеваний скрываются редкие наследственные коагулопатии, в том числе наследственный дефицит фактора свертывания крови V (FV).

Цель

Цель: описание клинических проявлений и выбора тактики лечения у больных с наследственным дефицитом FV.

Основные сведения

Основные сведения. Представлен обзор литературы и трех клинических наблюдений за больными с наследственным дефицитом FV. Обсуждаются вопросы дифференциальной диагностики наследственных коагулопатий, поскольку от точного диагноза зависит выбор гемостатической терапии. Больные с наследственным дефицитом FV требуют постоянного наблюдения гематологом с целью контроля спонтанного или индуцированного геморрагического синдрома, проведения гемостатической терапии при оперативных вмешательствах, беременности, родах.

Конфликт интересов

Конфликт интересов: авторы заявляют об отсутствии конфликта интересов.

Финансирование

Финансирование: исследование не имело спонсорской поддержки.

Introduction

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

Aim. To describe the clinical manifestations and treatment of congenital factor V defi ciency.

General findings

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

Conflict of interest: the authors declare no conflict of interest.

Financial disclosure

Financial disclosure: the study had no sponsorship.

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The authors declare that there are no conflicts of interest present.