Terapevticheskii arkhivTerapevticheskii arkhivТерапевтический архив0040-36602309-5342LLC Obyedinennaya Redaktsiya6029910.26442/00403660.2020.12.200508ArticlesСтатьиResearch ArticleDiagnosis of thrombotic thrombocytopenic purpuraДиагностика тромботической тромбоцитопенической пурпурыhttps://orcid.org/0000-0001-8818-8949GalstyanG. M.ГалстянГеннадий Мартинович
Russian Federation

д.м.н., зав. отд-нием

gengalst@gmail.comhttps://orcid.org/0000-0002-8141-9422KlebanovaE. E.КлебановаЕлизавета Евгеньевна
Russian Federation

врач отд-ния реанимации и интенсивной терапии

gengalst@gmail.comNational Research Center for HematologyФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России1512202092122072170702202107022021Copyright ©; 2020, Consilium MedicumCopyright ©; 2020, ООО "Консилиум Медикум"2020Consilium MedicumООО "Консилиум Медикум"https://creativecommons.org/licenses/by-nc-sa/4.0https://ter-arkhiv.ru/0040-3660/article/view/60299

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease, disease, characterised by microangiopathic hemolytic anaemia, consumption thrombocytopenia, and organ dysfunction. The pathogenesis of TTP is attributed to the deficiency in the activity of the metalloproteinase ADAMTS13, specific von Willebrand factor cleaving protease. TTP is suspected when detecting microangiopathic hemolytic anemia, thrombocytopenia, damage to various organs. Diagnosis of TTP is confirmed by the detection of ADAMTS13 activity in plasma less than 10%. Plasma samples for the study of ADAMTS13 activity should be taken before the start of plasma transfusions or plasma exchange. In patients with severe ADAMTS-13 deficiency autoantibodies anti-ADAMTS13 and inhibitor ADAMTS13 should be investigated. Anti-ADAMTS13 antibodies belonging to IgG not always have inhibitory effects. The inhibitory effect of anti-ADAMTS13 antibodies is confirmed by mixing test. All patients with the first established diagnosis of TTP should be examined for mutations of the ADAMTS13 gene.

Тромботическая тромбоцитопеническая пурпура (ТТП) – редкое угрожающее жизни заболевание, характеризующееся микроангиопатической гемолитической анемией, тромбоцитопенией потребления и органной дисфункцией. В основе патофизиологии ТТП лежит дефицит металлопротеиназы ADAMTS13, расщепляющей фактор фон Виллебранда. Предположительный диагноз ТТП устанавливают на основании выявления микроангиопатической гемолитической анемии, тромбоцитопении, поражения различных органов. Диагноз ТТП подтверждается обнаружением активности ADAMTS13 в плазме менее 10%. Образцы плазмы на исследование активности ADAMTS13 должны быть взяты до начала трансфузий плазмы либо плазмообмена. При выявлении низкой активности ADAMTS13 необходимо обследовать на наличие ингибитора ADAMTS13 иммуноферментным методом либо с помощью теста смешивания. Анти-ADAMTS13-антитела, относящиеся к иммуноглобулинам класса G, не всегда обладают ингибирующим действием. Ингибирующее действие анти-ADAMTS13-антител подтверждается в тесте смешивания. Впервые выявленные пациенты с ТТП должны быть обследованы на наличие мутаций гена ADAMTS13.

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