Наследcтвенный сочетанный дефицит факторов свертывания крови V и VIII
УДК 616.151.514:616-005
Аннотация
Резюме. Наследственный сочетанный дефицит факторов свертывания крови V (FV) и VIII (FVIII) является редкой наслед- ственной коагулопатией. Недостаточность публикаций об этом заболевании в Российской Федерации создает препятствие оказанию помощи таким больным. В большинстве случаев диагностика наследственного сочетанного дефицита FV и FVIII и назначение необходимой терапии являются запоздалыми, что ухудшает прогноз заболевания и качество жизни больных. В обзоре представлены данные о распространенности, этиопатогенезе, наследовании, клинических проявлениях и лечении наследcтвенного сочетанного дефицита FV и FVIII.
Для цитирования: Яковлева Е.В., Зозуля Н.И. Наследcтвенный сочетанный дефицит факторов свертывания крови V и VIII. Тромбоз, гемостаз и реология. 2023;(4):4–10.
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Ключевые слова
наследственные коагулопатии, наследcтвенный сочетанный дефицит факторов V и VIII свертывания крови, геморрагический синдром