nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2020-7-4-71-76nodgo-670Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSТерапия редких коагулопатийTreatment of rare bleeding disordershttps://orcid.org/0000-0003-4555-9337ФлоринскийД. Б.FlorinskiyD. B.

Флоринский Дмитрий Борисович - врач-ординатор.117997, Москва, ул. Саморы Машела, 1.

Resident in Pediatrics Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.1 Samory Mashela St., Moscow, 117997.

mitia94@yandex.ruhttps://orcid.org/0000-0003-4384-6754ЖарковП. А.ZharkovP. A.

Доктор медицинских наук, врач-гематолог консультативного отделения, врач-педиатр стационара кратковременного лечения, руководитель группы исследования гемостаза.117997, Москва, ул. Саморы Машела, 1.

Dr. of Sci. (Med.), Hematologist Advisory Unit, Short-Term Hospital Pediatrician, Head of the Hemostasis Research Group of Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology, Ministry of Health of Russia.1 Samory Mashela St., Moscow, 117997.

pavel.zharkov@fccho-moscow.ruНМИЦ ДГОИ им. Дмитрия Рогачева Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaRussian Federation202007012021747176Copyright © Флоринский Д.Б., Жарков П.А., 20212021Флоринский Д.Б., Жарков П.А.Florinskiy D.B., Zharkov P.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nodgo.org/jour/article/view/670

Редкие коагулопатии включают в себя наследственные дефициты фибриногена, факторов (F) II, FV, FVII, FX, FXI, FXII и FV + FVIII, а также комбинированный дефицит витамина К-зависимых факторов. Какие-то из данных дефицитов являются более изученными в связи с большим числом пациентов, какие-то — крайне редкими, поэтому на данном этапе представляется достаточно сложной проблема выработки универсального подхода к терапии и профилактике. Задачей данного обзора было оценить варианты лечения и возможности профилактики данных дефицитов.

Rare bleeding disorders include inherited deficiencies of fibrinogen, factors (F)II, FV, FVII, FX, FXI, FXII, and FV + FVIII, as well as a multiple deficiency of vitamin K-dependent coagulation factors. Some of these deficiencies are more studied, due to the large number of patients, some are extremely rare, and so at this stage it is quite difficult for them to develop a universal approach to therapy and prophylactic treatment. The purpose of this review was to evaluate treatment options for these deficiencies.

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