Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children85110.24287/1726-1708-2024-23-1-200-210LITERATURE REVIEWОБЗОР ЛИТЕРАТУРЫModern aspects of hemophilia A diagnosisСовременные аспекты диагностики гемофилии Аhttps://orcid.org/0000-0001-5209-2099PoletaevA. V.ПолетаевА. В.
Russian Federation

Alexander V. Poletaev, Acting Head of the Laboratory

Laboratory of Clinical Hemostasis

117997; 1 Samory Mashela St.; Moscow

Александр Владимирович Полетаев, и. о. заведующего лабораторией

лаборатория клинического гемостаза

117997; ул. Саморы Машела, 1; Москва

poletaev_alexandr@mail.ruhttps://orcid.org/0000-0002-7534-3863SereginaE. A.СерёгинаЕ. А.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0003-4384-6754ZharkovP. A.ЖарковП. А.
Russian Federation

Moscow

Москва

The Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава РоссииCenter for Theoretical Problems of Physical and Chemical PharmacologyФГБУН «Центр теоретических проблем физико-химической фармакологии» РАН190420242312002101804202418042024Copyright ©; 2024, «D. Rogachev NMRCPHOI»Copyright ©; 2024, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2024«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/851

   The evolution of hemophilia treatment is rapidly developing. Both new factor replacement and non-factor therapy have appeared in recent years. One of the most important problems of factor replacement therapy is the relatively short half-life of coagulation factor VIII (FVIII), with an average of about 8–12 hours in adults, ranging in individual patients between 6 and 24 hours, and even shorter in younger children. This forces patients, especially children, to administer the drug quite often (3–4 times a week), reducing the quality of life and adherence to treatment. The appearance of recombinant FVIII products with an increased half-life allows to reduce the number of infusions per week, improving the quality of life of patients without compromising the safety and efficacy of treatment. However, the structure of these products leads to the changes in the results of laboratory tests of FVIII activity carried out to monitor the efficacy of treatment. In this article, we will consider the current methods of laboratory control of products with an increased half-life of FVIII currently available in Russia. We want to assess the discrepancy between the one-stage clotting method and chromogenic method for each FVIII product, as well as the laboratory's capabilities in monitoring non-factor and combined therapy for hemophilia A.

   В последние годы происходит стремительная эволюция лечения гемофилии А, появляется все больше препаратов как факторной, так и нефакторной терапии. Одной из важных проблем заместительной факторной терапии является относительно короткий период полувыведения фактора свертывания крови VIII (FVIII), составляющий в среднем 8–12 ч, что вынуждает пациентов, особенно детского возраста, вводить препарат достаточно часто (3–4 раза/нед), снижая качество жизни и приверженность к терапии. Появление рекомбинантных препаратов FVIII с увеличенным периодом полувыведения позволяет уменьшить количество инфузий, улучшая качество жизни пациентов без снижения безопасности и эффективности. Однако особенности структуры данных препаратов приводят к изменению результатов лабораторных исследований активности FVIII, проводимых для контроля эффективности терапии. В данной статье мы рассмотрим современные методы лабораторного контроля доступных на сегодняшний день препаратов с увеличенным периодом полужизни FVIII в России, оценим степень расхождения между одностадийным клоттинговым и хромогенным методами для каждого препарата, а также возможности лаборатории в мониторинге нефакторной и сочетанной терапии гемофилии А.

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