bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2025-70-4-498-510bloodjour-694Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESВрожденная тромботическая тромбоцитопеническая пурпура у взрослых: проявления и лечениеCongenital thrombotic thrombocytopenic purpura in adults: Manifestations and treatmenthttps://orcid.org/0000-0001-8818-8949ГалстянГ. М.GalstyanG. M.

Галстян Геннадий Мартинович, доктор медицинских наук, заведующий отделом реанимации и интенсивной терапии

125167, г. Москва

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

125167, Moscow

gengalst@gmail.comhttps://orcid.org/0000-0002-8141-9422КлебановЕ. Е.KlebanovaE. E.

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

125167, г. Москва

Elizaveta E. Klebanova, anesthesiologist-resuscitator of the Resuscitation and Intensive Care Department

125167, Moscow

klebanova.liza@yandex.ruhttps://orcid.org/0000-0001-8964-220XПозняковаЮ. М.PoznyakovaY. M.

Познякова Юлия Михайловна, ведущий специалист лаборатории генной инженерии

125167, г. Москва

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

125167, Moscow 

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

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

125167, г. Москва

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

125167, Moscow

pshenichnikovaolesya@gmail.comhttps://orcid.org/0000-0003-1492-1735МамлееваС. Ю.MamleevaS. Yu.

Мамлеева Светлана Юрьевна, заведующая экспресс-лабораторией отделения реанимации и интенсивной терапии

125167, г. Москва

Svetlana Yu. Mamleeva, Head of the Express-Laboratory, of the Resuscitation and Intensive Care Department

125167, Moscow

maml.s-yur@yandex.ruhttps://orcid.org/0000-0002-2905-5959ПурлоН. В.PurloN. V.

Пурло Наталья Владимировна, кандидат медицинских наук, нефролог отдела анестезиологии и реанимации

125167, г. Москва

Natalia V. Purlo, Cand. Sci. (Med.), Nephrologist of Dialysis group in the Resuscitation and Intensive Care Department

125167, Moscow

n_purlo@mail.ruhttps://orcid.org/0009-0005-7449-3930ИпатоваН. Г.IpatovaN. G.

Ипатова Наталья Геннадьевна, заведующая гематологическим отделением, гематолог

426039, г. Ижевск

Natalia G. Ipatova, Head of the Hematology Department-hematologist

426039, Izhevsk

ipatova07@inbox.ruhttps://orcid.org/0000-0002-1890-4492СуринВ. Л.SurinV. L.

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

125167, г. Москва

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

125167, Moscow

vadsurin@mail.ruФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for HematologyRussian FederationБУЗ Удмуртской Республики «Первая республиканская клиническая больница» Министерства здравоохранения Удмуртской РеспубликиРоссияFirst Republican Clinical HospitalRussian Federation202521122025704498510Copyright © Галстян Г.М., Клебанов Е.Е., Познякова Ю.М., Пшеничникова О.С., Мамлеева С.Ю., Пурло Н.В., Ипатова Н.Г., Сурин В.Л., 20252025Галстян Г.М., Клебанов Е.Е., Познякова Ю.М., Пшеничникова О.С., Мамлеева С.Ю., Пурло Н.В., Ипатова Н.Г., Сурин В.Л.Galstyan G.M., Klebanova E.E., Poznyakova Y.M., Pshenichnikova O.S., Mamleeva S.Y., Purlo N.V., Ipatova N.G., Surin V.L.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/694Введение

Введение. Врожденная тромботическая тромбоцитопеническая пурпура (вТТП) — редкая тромботическая микроангиопатия, причиной которой является мутация гена ADAMTS13, в результате чего возникает дефицит фермента ADAMTS13. Может манифестировать как в детском, так и во взрослом возрасте.

Цель — анализ проявлений и течения вТТП у взрослых больных.

Материалы и методы

Материалы и методы. У всех больных с подозрением на тромботическую тромбоцитопеническую пурпуру (ТТП) исследовали концентрации гемоглобина и тромбоцитов крови, прямую пробу Кумбса, активность лактатдегидрогеназы (ЛДГ) и  ADAMTS13, ингибитор ADAMTS13. При активности АDAMTS13 < 10% и  отсутствии ингибитора ADAMTS13 исследовали концентрацию антигена АDAMTS13, мутации гена ADAMTS13. Диагноз вТТП устанавливали при отсутствии ингибитора ADAMTS13 и обнаружении мутаций гена ADAMTS13.

Результаты

Результаты. Среди 115 взрослых больных ТТП, у 11 (9,5%) человек (10 женщин и 1 мужчина) была диагностирована вТТП. У всех больных выявлены мутации гена ADAMTS13. Манифестация заболевания — в возрасте от 20 до 33 лет (медиана 22 года). У 9 из 10 женщин дебют заболевания был ассоциирован с беременностью. При манифестации ТТП у всех была тромбоцитопения ((4–31)×109 /л, медиана 14×109 /л), анемия (гемоглобин 67 г/л), повышение активности ЛДГ (медиана 1300 Ед/л), низкая активность ADAMTS13 (медиана 2%), низкая концентрация антигена ADAMTS13 (медиана 0,008 МЕ/мл). Неврологическая симптоматика была у 5 (45%) из 11 больных, у 2 из 11 больных развилась хроническая почечная недостаточность. При достижении клинической ремиссии самочувствие всех больных было удовлетворительным, гемоглобин, тромбоциты крови, активность ЛДГ были в пределах нормы, однако сохранялась низкая активность ADAMTS13 (0–18%, медиана 7,5%). Лечение при обострении — трансфузии плазмы, у 8 больных выполняли плазмообмены. Профилактически большинство больных получали плазму в дозе 15 мл/кг каждые 3–4 недели. У 2 больных начато лечение рекомбинантным ADAMTS13 (rADAMTS13) в дозе 40 МЕ/кг внутривенно раз в  2  недели. Терапию переносят удовлетворительно, отмечается прирост тромбоцитов крови после введения препарата.

Заключение

Заключение. вТТП может манифестировать во взрослом возрасте. В состоянии ремиссии все больные вТТП нуждаются в наблюдении и профилактическом лечении.

Introduction

Introduction. Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare thrombotic microangiopathy that occurs due to a congenital deficiency of the ADAMTS13 enzyme. It can manifest both in childhood and in adulthood.

Aim

Aim: to analyze the manifestations and course of cTTP in adult patients. Materials and methods. Hemoglobin and platelet concentrations, a direct Coombs test, lactate dehydrogenase (LDH), ADAMTS13 activity, and ADAMTS13 inhibitor were studied in patients with suspected thrombotic thrombocytopenic purpura (TTP). In patients with ADAMTS13 activity< 10% and the absence of an ADAMTS13 inhibitor, the concentration of the ADAMTS13 antigen and mutations of the ADAMTS13 gene were studied. cTTP was diagnosed in the absence of ADAMTS13 inhibitor and the detection of mutations in ADAMTS13 gene.

Results

Results. cTTP was diagnosed in 11 (9.5 %) patients (10 women, 1 man) among 115 adult patients with TTP. Mutations of ADAMTS13 gene were detected in all patients. Disease onset occurred between the ages of 20 and 33 years 33 (median 22 years). In 9 out of 10 women, the onset of the disease was associated with pregnancy. During TTP manifestation all patients had thrombocytopenia ((4–31)×109 /L, median 14×109 /L), anemia (hemoglobin 67 g/L), increased LDH activity (median 1300 U/L), low ADAMTS13 activity (median 2 %), and low ADAMTS13 antigen concentration (median 0.008 IU/ml). Neurological symptoms were present in 5 (45 %) of the 11 patients, and 2 of the 11 patients developed chronic renal failure. In clinical remission, all patients had normal ranges of hemoglobin, platelets and LDH activity, however ADAMTS13 activity remained low (0–18 %, median 7.5 %). Treatment during exacerbation included plasma transfusions; 8 patients underwent plasma exchange. Prophylactically, most patients received plasma at a dose of 15 mL/kg every 3–4 weeks. Three patients were treated with recombinant ADAMTS13 at a dose of 40 IU/kg intravenously once every 2 weeks. The therapy was well tolerated, and there was an increase of platelet level after drug administration.

Conclusion

Conclusion. cTTP can manifest in adulthood. In remission, cTTP patients need laboratory monitoring and preventive treatment.

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