medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/2079-701X-2021-3-98-105medsovet-6079Research ArticleПРЕГРАВИДАРНАЯ ПОДГОТОВКА И БЕРЕМЕННОСТЬPREGRAVID PREPARATION AND PREGNANCYНовое в дифференциальной диагностике различных вариантов тромботических микроангиопатий в акушерствеNew in the differential diagnosis of different variants of thrombotic microangiopathies in obstetricshttps://orcid.org/0000-0002-6125-590XКирсановаТ. В.KirsanovaT. V.

Кирсанова Татьяна Валерьевна - кандидат медицинских наук, старший научный сотрудник отделения репродуктивной гематологии и клинической гемостазиологии.

117997, Москва, ул. Академика Опарина, д. 4

Tatyana V. Kirsanova - Cand. Sci. (Med.), Senior Researcher, Department of Reproductive Hematology and Clinical Hemostasiology.

4, Academician Oparin St., Moscow, 117997

a_tatya@mail.ruhttps://orcid.org/0000-0001-9827-1922ВиноградоваМ. А.VinogradovaM. A.

Виноградова Мария Алексеевна - кандидат медицинских наук, заведующая отделением репродуктивной гематологии и клинической гемостазиологии.

117997, Москва, ул. Академика Опарина, д. 4

Mariya A. Vinogradova - Cand. Sci. (Med.), Head of the Department of Reproductive Hematology and Clinical Hemostasiology

4, Academician Oparin St., Moscow, 117997

mary-grape@ya.ruНациональный медицинский исследовательский центр акушерства, гинекологии и перинатологии им. академика В.И. КулаковаРоссияKulakov National Medical Research Center of Obstetrics, Gynecology and PerinatologyRussian Federation2021150420210398105Copyright © Кирсанова Т.В., Виноградова М.А., 20212021Кирсанова Т.В., Виноградова М.А.Kirsanova T.V., Vinogradova M.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-sovet.pro/jour/article/view/6079

Все варианты тромботической микроангиопатии в акушерской практике развиваются лавинообразно и требуют быстрого решения о выборе тактики лечения. Несмотря на то что все эти заболевания имеют схожие признаки, характерные для синдрома ТМА в целом, они являются отдельными заболеваниями с разной этиологией и патогенезом. В связи с тем, что проявления ТМА практически идентичны при различных болезнях, все они могут имитировать преэклампсию, а терапевтические подходы различаются, проведение дифференциальной диагностики представляется крайне важным и зачастую определяющим прогноз мероприятием.

Описано проведение дифференциально диагностического поиска между различными вариантами ТМА, которые могут развиваться при беременности, т. к. от правильного диагноза зачастую зависит прогноз жизни. Рассмотрены основные отличия преэклампсии/HELLP-синдрома от таких жизнеугрожающих состояний, как атипичный гемолитико-уремический синдром, тромботическая тромбоцитопеническая пурпура и катастрофический антифосфолипидный синдром. Предложены алгоритмы диагностического поиска. Обращено внимание на то, что существующие алгоритмы диагностики тяжелой ТМА до получения результатов ADAMTS 13, основанные на выраженности тромбоцитопении и азотемии, а также балльные шкалы во время беременности могут служить ориентиром, но не определяющим диагноз признаком.

Ключом к дифференциальной диагностике является влияние родоразрешения на регресс ТМА: если после родоразрешения выраженность гемолиза и тромбоцитопении уменьшается, то можно говорить о «чистых» преэклампсии или HELLP-синдроме. Если сохраняются или нарастают, то следует думать о тромбоцитопенической пурпуре и аГУС. При уровне ADAMTS 13 менее 10% устанавливается диагноз «ТТП», при ADAMTS 13 более 10-20% пациент попадает в «серую зону» и требуется повторное исследование фермента. Если имеются признаки фульминантно развившейся полиорганной недостаточности, особенно у пациента с исходным подозрением на АФС, то можно предполагать КАФС. Для его подтверждения необходимы высокие титры АФА. Кроме того, ТМА могут вызвать: сепсис и различные ургентные акушерские состояния, осложняющиеся развитием ДВС.

All variants of thrombotic microangiopathy (TMA) in obstetric practice develop in an avalanche-like manner and require a rapid decision on the choice of treatment tactics. Although all these diseases have similar features, typical of the TMA syndrome as a whole, they are separate diseases with differing etiology and pathogenesis. Because the manifestations of TMA are almost identical in the different diseases, they may all imitate pre-eclampsia and the therapeutic approaches differ, a differential diagnosis is essential and often determines the prognosis.

The differential diagnostic search between the different TMA variants that can develop in pregnancy is described, as the prognosis of life often depends on the correct diagnosis. The main differences between pre-eclampsia/HELLP syndrome and life-threatening conditions such as atypical hemolytic-uremic syndrome, thrombotic thrombocytopenic purpura and catastrophic antiphospholipid syndrome are considered. Algorithms of diagnostic search are proposed. Attention is drawn to the fact that existing algorithms for the diagnosis of severe TMA prior to ADAMTS 13 results, based on the severity of thrombocytopenia and azotemia as well as scoring during pregnancy can serve as a guide, but not as a defining feature of the diagnosis.

The key to the differential diagnosis is the effect of delivery on the regression of TMA: if hemolysis and thrombocytopenia decrease after delivery, one can speak of ‘pure’ pre-eclampsia (PE) or HELLP-syndrome. If persistent or increasing, thrombotic thrombocytopenic purpura (TTP) and aHUS should be considered. With an ADAMTS 13 level of less than 10%, a diagnosis of TTP is made. With ADAMTS 13 over 10-20%, the patient falls into the «grey zone» and a repeat enzyme test is required. If there are signs of fulminant multiple organ failure, especially in a patient with initial suspicion of APS, CAPS can be suspected. To confirm it, high titres of APA are required. In addition, TMA may cause: sepsis and various urgent obstetric conditions complicated by the development of DIC.

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