bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2022-67-1-42-61bloodjour-336Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESТромботическая тромбоцитопеническая пурпура у беременных: родоразрешать нельзя прерывать. Где поставить запятую?Thrombotic thrombocytopenic purpura in pregnancy. Giving birth or not giving birth, that is the questionhttps://orcid.org/0000-0001-8818-8949ГалстянГ. М.GalstyanG. M.

Галстян Геннадий Мартинович, доктор медицинских наук, заведующий отделением реанимации и интенсивной терапии; профессор кафедры анестезиологии и реаниматологии 

125167, Москва; 117997, Москва

Gennadiy M. Galstyan, Dr. Sci. (Med.), Head of the Resuscitation and Intensive Care Department; professor of the Department of Anesthesiology and Intensive Care 

125167, Moscow; 117997, Moscow

gengalst@gmail.comhttps://orcid.org/0000-0002-2206-1002ШмаковР. Г.ShmakovR. G.

Шмаков Роман Георгиевич, доктор медицинских наук, профессор РАН, главный внештатный специалист по акушерству Министерства здравоохранения Российской Федерации, директор института акушерства 

117997, Москва

Roman G. Shmakov, Dr. Sci. (Med.), professor of the RAS, Chief Obstetric Specialist of the Ministry of Health of the Russian Federation, Director of the Institute of Obstetrics 

117997, Moscow

r_shmakov@oparina4.ruhttps://orcid.org/0000-0002-8141-9422КлебановаЕ. Е.KlebanovaЕ. Е.

Клебанова Елизавета Евгеньевна, врач анестезиолог-реаниматолог отделения реанимации и интенсивной терапии 

125167, Москва

Elizaveta E. Klebanova, Physician of the Resuscitation and Intensive Care Department 

125167, Moscow

klebanova.liza@yandex.ruhttps://orcid.org/0000-0002-4827-8947ТроицкаяВ. В.TroitskayaV. V.

Троицкая Вера Витальевна, кандидат медицинских наук, заведующая отделением высокодозной интенсивной химиотерапии гемобластозов и депрессий кроветворения 

125167, Москва

Vera V. Troitskaya, Cand. Sci. (Med.), Head of the Department of Intensive High-dose Chemotherapy of Hemoblastosis and Bone Marrow Depression 

125167, Moscow

troitskaya.v@blood.ruhttps://orcid.org/0000-0002-9877-0796ДвирныкВ. Н.DvirnykV. N.

Двирнык Валентина Николаевна, кандидат медицинских наук, заведующая центральной клинико-диагностической лаборатории 

125167, Москва

Valentina N. Dvirnyk, Cand. Sci. (Med.), Head of the Centralized Clinical and Diagnostic Laboratory 

125167, Moscow

dvirnyk.v@blood.ruhttps://orcid.org/0000-0002-1890-4492СуринВ. Л.SurinV. L.

Сурин Вадим Леонидович, старший научный сотрудник, и.о. заведующего лабораторией генной инженерии 

125167, Москва

Vadim L. Surin, Senior Researcher, Laboratory of Genetic Engineering 

125167, Moscow

vadsurin@mail.ruhttps://orcid.org/0000-0001-5752-8146ПшеничниковаО. С.PshenichnikovaO. S.

Пшеничникова Олеся Сергеевна, кандидат биологических наук, старший научный сотрудник лаборатории генной инженерии 

125167, Москва

Olesya S. Pshenichnikova, Senior Researcher, Laboratory of Genetic Engineering 

125167, Moscow

pshenichnikovaolesya@gmail.comhttps://orcid.org/0000-0001-8964-220XПозняковаЮ. М.PozdnyakovaYu. M.

Познякова Юлия Михайловна, ведущий специалист лаборатории генной инженерии 

125167, Москва

Yuliya M. Pozdnyakova, Leading Specialist, Laboratory of Genetic Engineering 

125167, Moscow

y.poznyakova@mail.ruhttps://orcid.org/0000-0002-1945-0154ПолушкинаЕ. С.PolushkinaE. S.

Полушкина Евгения Сергеевна, кандидат медицинских наук, старший научный сотрудник, институт акушерства 

117997, Москва

Evgeniya S. Polushkina, Cand. Sci. (Med.), Senior Researcher, Institute of Obstetrics 

117997, Moscow

epolushkina@mail.ruhttps://orcid.org/0000-0002-9684-5045ГапоноваТ. В.GaponovaT. V.

Гапонова Татьяна Владимировна, кандидат медицинских наук, заведующая отделом процессинга клеток крови и криоконсервирования 

125167, Москва

Tatyana V. Gaponova, Cand Sci. (Med), Head of Department of Cells Processing and Cryopreservation 

125167, Moscow

gaponova.tatj@yandex.ruhttps://orcid.org/0000-0003-1492-1735МамлееваС. Ю.MamleevaS. Yu.

Мамлеева Светлана Юрьевна, врач экспресс-лаборатории 

125167, Москва

Svetlana Yu. Mamleeva, Physician of the Express-Laboratory 

125167, Moscow

maml.s-yur@yandex.ruhttps://orcid.org/0000-0001-8382-9671ПыреговА. В.PyregovA. V.

Пырегов Алексей Викторович, доктор медицинских наук, директор института анестезиологии-реаниматологии и трансфузиологии 

117997, Москва

Alexey V. Pyregov, Dr. Sci. (Med.), CEO of the Institute of Anesthesiology, Reanimatology and Transfusiology 

117997, Moscow

pyregov@mail.ruhttps://orcid.org/0000-0001-9847-5765РогачевскийО. В.RogachevskiyО. V.

Рогачевский Олег Владимирович, доктор медицинских наук, заведующий отделением экстракорпоральных методов лечения и детоксикации Института анестезиологии-реаниматологии и трансфузиологии 

117997, Москва

Oleg V. Rogachevskiy, Dr. Sci. (Med.), Head of the Department of Extracorporal Treatment Methods and Detoxication 

117997, Moscow

orogachevskiy@gmail.comhttps://orcid.org/0000-0002-5056-9540СысоеваЕ. П.SysoevaE. P.

Сысоева Елена Павловна, кандидат медицинских наук, старший научный сотрудник отделения орфанных заболеваний 

125167, Москва

Elena P. Sysoeva, Cand. Sci. (Med.), Senior Researcher, Department of Orphan Diseases 

125167, Moscow

sysoeva.e@blood.ruhttps://orcid.org/0000-0002-0977-215XЦветаеваН. В.TsvetaevaN. V.

Цветаева Нина Валентиновна, кандидат медицинских наук, старший научный сотрудник отделения орфанных заболеваний 

125167, Москва

Nina V. Tsvetaeva, Cand. Sci. (Med.), Senior Researcher of the Department of Orphan Diseases 

125167, Moscow

tsvetaeva51@gmail.comФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской Федерации; ФГБУ «Национальный медицинский исследовательский центр акушерства, гинекологии и перинатологии имени академика В.И. Кулакова» Министерства здравоохранения Российской ФедерацииРоссияNational Research Center for Hematology; National Medical Research Center for Obstetrics, Gynecology and Perinatology named after Academician V.I. KulakovRussian FederationФГБУ «Национальный медицинский исследовательский центр акушерства, гинекологии и перинатологии имени академика В.И. Кулакова» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for Obstetrics, Gynecology and Perinatology named after Academician V.I. KulakovRussian FederationФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Research Center for HematologyRussian Federation2022080420226714261Copyright © Галстян Г.М., Шмаков Р.Г., Клебанова Е.Е., Троицкая В.В., Двирнык В.Н., Сурин В.Л., Пшеничникова О.С., Познякова Ю.М., Полушкина Е.С., Гапонова Т.В., Мамлеева С.Ю., Пырегов А.В., Рогачевский О.В., Сысоева Е.П., Цветаева Н.В., 20222022Галстян Г.М., Шмаков Р.Г., Клебанова Е.Е., Троицкая В.В., Двирнык В.Н., Сурин В.Л., Пшеничникова О.С., Познякова Ю.М., Полушкина Е.С., Гапонова Т.В., Мамлеева С.Ю., Пырегов А.В., Рогачевский О.В., Сысоева Е.П., Цветаева Н.В.Galstyan G.M., Shmakov R.G., Klebanova Е.Е., Troitskaya V.V., Dvirnyk V.N., Surin V.L., Pshenichnikova O.S., Pozdnyakova Y.M., Polushkina E.S., Gaponova T.V., Mamleeva S.Y., Pyregov A.V., Rogachevskiy О.V., Sysoeva E.P., Tsvetaeva N.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.htjournal.ru/jour/article/view/336Введение

Введение. Беременность  — один из  наиболее частых триггеров наследственной и  приобретенной форм тромботической тромбоцитопенической пурпуры (ТТП).

Цель — разработать тактику лечения беременных женщин с ТТП.

Материалы и методы

Материалы и методы. Изучены течение и исходы 12 беременностей у 10 женщин, больных ТТП (4 — врожденной ТТП и 6 — приобретенной ТТП). Исследовали активность ADAMTS13, наличие ингибитора ADAMTS13, генетических мутаций, осложнения во время беременности и при родах, тактику лечения ТТП, исходы беременностей.

Результаты

Результаты. ТТП была ассоциирована с  беременностью в  55,5  % всех случаев заболевания ТТП у  женщин. При 5 беременностях, при которых диагноз ТТП был известен до беременности либо установлен на ранних сроках, проводили лечение ТТП на протяжении всей беременности. При врожденной форме выполняли трансфузии плазмы раз в 2 недели до 20-й недели беременности. Если сохранялась тромбоцитопения < 150 × 109 /л, а также после 20-й недели трансфузии плазмы проводили еженедельно, цель — достижение клинической ремиссии. При приобретенной ТТП проводили терапию глюкокортикостероидами и  плазмообменами, в  1  случае  — ритуксимабом, цель  — достижение клинической ремиссии и активности ADAMTS13 > 20 %. В этой группе беременных было выполнено 1 кесарево сечение, в 4 случаях — вагинальные роды, родились 5 детей, в среднем 7,5 балла по шкале Апгар. При 7 беременностях, при которых ТТП впервые манифестировала на поздних сроках, диагноз устанавливался поздно, профилактические и лечебные мероприятия до родов не проводили. В этой группе было выполнено 2 прерывания беременности, 5 оперативных родоразрешений, отмечено 3 случая преэклампсии, 3 случая острого нарушения мозгового кровообращения, 1  внутрибрюшное кровотечение, 1  случай острой почечной недостаточности, двум женщинам проводили искусственную вентиляцию легких, был 1  случай антенатальной гибели плода, родились 4 детей, которых оценили по шкале Апгар в среднем в 5 баллов.

Заключение

Заключение. Своевременно установленный диагноз, проводимые лечебные и  профилактические мероприятия позволяют избежать осложнений во время родов у беременных с ТТП.

Introduction

Introduction. Pregnancy is one of the most frequent triggers of congenital and acquired forms of thrombotic thrombocytopenic purpura (TTP).

Aim — to develop tactics for the treatment of pregnant women with TTP.

Results

Results. TTP was associated with pregnancy in 55.5 % of all cases of TTP in women. In 5 pregnancies in which the diagnosis of TTP was known before pregnancy, or established in the early stages, TTP was treated throughout the pregnancy. In the congenital form, plasma transfusions were performed once every two weeks until the  20th  week of pregnancy, or  weekly, if thrombocytopenia < 150 × 109 /L persisted. Plasma transfusions were performed weekly after the 20th week with the goal of achieving clinical remission. With acquired TTP, glucocorticosteroids and plasma exchanges were used as  treatment, in 1 case — rituximab, with the aim of achieving a clinical remission and an ADAMTS13 activity > 20 %. In this group of pregnant women, 1 caesarean section was performed and there were 4 cases of vaginal deliveries, with a total of 5 children being born with an average Apgar score of 7.5. In 7 pregnancies in which TTP fi rst manifested late, leading to a delayed diagnosis, preventive and curative measures were not carried out before delivery. In this group there were 2 abortions of pregnancy, 5 surgical deliveries, 3 cases of preeclampsia, 3 acute cerebral circulatory disorders, 1 intraabdominal bleeding, 1 case of acute renal failure, with two women undergoing mechanical ventilation. There was 1 case of antenatal fetal death, with a total of 4 children being born, who were assessed on the Apgar scale with an average of 5 points.

Conclusion

Conclusion. Timely diagnosis as well as ongoing therapeutic and preventive measures help to avoid complications during childbirth in pregnant women with TTP.

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