bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2023-68-3-317-334bloodjour-475Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESИммунная тромбоцитопеническая пурпура и тромботическая тромбоцитопеническая пурпура — сложности и ошибки диагностикиImmune thrombocytopenic purpura and thrombotic thrombocytopenic purpura — diagnostic difficulties and errorshttps://orcid.org/0000-0001-8818-8949ГалстянГ. М.GalstyanG. M.

Геннадий Мартинович Галстян, доктор медицинских наук, заведующийотделением

отделение реанимации и интенсивной терапии

125167

Москва

Gennadiy M. Galstyan, Dr. Sci. (Med.), Head of the Department

Resuscitation and Intensive Care Department

125167

Moscow

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

Елизавета Евгеньевна Клебанова, анестезиолог-реаниматолог

отделение реанимации и интенсивной терапии

125167

Москва

Elizaveta E. Klebanova, Physician

Resuscitation and Intensive Care Department

125167

Moscow

klebanova.liza@yandex.ruМамлееваС. Ю.MamleevaS. Yu.

Светлана Юрьевна Мамлеева, заведующая экспресс-лабораторией

отделение реанимации и интенсивной терапии

125167

Москва

Svetlana Yu. Mamleeva, Head of the Express-Laboratory

125167

Moscow

maml.s-yur@yandex.ruhttps://orcid.org/0000-0002-8406-0240ГржимоловскийА. В.GrzhimolovskiiA. V.

Александр Вадимович Гржимоловский, кандидат медицинских наук, заведующий блоком

операционный блок

125167

Москва

Alexsandr V. Grzhimolovskii, Cand. Sci. (Med.), Head of the block

surgery block

125167

Moscow

grzhymolovsky.a@blood.ruhttps://orcid.org/0000-0002-1082-8659КовригинаА. М.KovriginaA. M.

Алла Михайловна Ковригина, доктор биологических наук, заведующая отделением

патологоанатомическое отделение

125167

Москва

Alla M. Kovrigina, Dr. Sci. (Biol.), Head of the Department

Pathological Department

125167

Moscow

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

Валентина Николаевна Двирнык, кандидат медицинских наук, заведующая лабораторий

лаборатория клинической лабораторной диагностики

125167

Москва

Valentina N. Dvirnyk, Cand. Sci. (Med.), Head of the Laboratory

Centralized Clinical and Diagnostic Laboratory

125167

Moscow

dvirnyk.v@blood.ruhttps://orcid.org/0000-0001-5905-7237БессмертныйД. К.BessmertnyyD. K.

Дмитрий Константинович Бессмертный, гематолог

отделение химиотерапии гемобластозов и депрессий кроветворения

125167

Москва

Dmitry K. Bessmertnyy, Physician

Department for Chemotherapy of Hematological malignancies and Hematopoietic Depressions

125167

Moscow

dmitry_bessmertnyy@mail.ruhttps://orcid.org/0000-0003-0934-6094ФидароваЗ. Т.FidarovaZ. T.

Залина Таймуразовна Фидарова, кандидат медицинских наук, заведующая отделением

отделение химиотерапии гемобластозов и депрессий кроветворения

125167

Москва

Zalina T. Fidarova, Cand. Sci. (Med.), Head of Department

Department for Chemotherapy of Hematological malignancies and Hematopoietic Depressions

125167

Moscow

zalinafidarova@mail.ruhttps://orcid.org/0000-0002-2119-3775МеликянА. Л.MelikyanA. L.

Анаит Левоновна Меликян, доктор медицинских наук, заведующая отделением

отделение стандартизации методов лечения

125167

Москва

Anahit L. Melikyan, Dr. Sci. (Med.), Head of Department

Department of Standardization of Treatment Methods

125167

Moscow

anoblood@mail.ruФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for HematologyRussian Federation202315112023683317334Copyright © Галстян Г.М., Клебанова Е.Е., Мамлеева С.Ю., Гржимоловский А.В., Ковригина А.М., Двирнык В.Н., Бессмертный Д.К., Фидарова З.Т., Меликян А.Л., 20232023Галстян Г.М., Клебанова Е.Е., Мамлеева С.Ю., Гржимоловский А.В., Ковригина А.М., Двирнык В.Н., Бессмертный Д.К., Фидарова З.Т., Меликян А.Л.Galstyan G.M., Klebanova E.E., Mamleeva S.Y., Grzhimolovskii A.V., Kovrigina A.M., Dvirnyk V.N., Bessmertnyy D.K., Fidarova Z.T., Melikyan A.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/475   Введение

   Введение. При диагностике впервые выявленной тромботической тромбоцитопенической пурпуры (ТТП) возможно ошибочно диагностировать другие заболевания, протекающие с тромбоцитопенией.

   Цель

   Цель: анализ ошибок и сложностей, возникающих при диагностике ТТП.

   Материалы и методы

   Материалы и методы. В исследование включены 54 больных (44 женщины и 10 мужчин) в возрасте от 18 до 83 лет (медиана 33 года), у которых был установлен диагноз ТТП с 2019 по 2023 гг. Анализировали анамнез, исходные показатели гемоглобина, тромбоцитов, эритроцитов, концентрации лактатдегидрогеназы (ЛДГ) и билирубина сыворотки, предварительно установленные диагнозы, проводимое лечение, исходы.

   Результаты

   Результаты. Лишь у 19 (35 %) больных первоначально сразу была заподозрена ТТП. Наиболее часто (у 12 из 54 больных) первоначально ошибочно диагностировали иммунную тромбоцитопению (ИТП). Еще у 3 больных ошибочно был заподозрен синдром Фишера — Эванса. В единичных случаях первоначально были заподозрены миелодиспластический синдром, системная красная волчанка / антифосфолипидный синдром, аутоиммунная гемолитическая анемия. Среди 15 женщин с дебютом ТТП во время беременности у 8 (53,3 %) первоначально предполагали HELLP-синдром. Умерли 12 (22,2 %) из 54 больных ТТП. У 11 из 12 умерших больных так и не было начато лечение ТТП, поскольку врачи, заподозрив ТТП, ожидали результатов исследования активности ADAMTS13. В то же время у больного с длительным течением ИТП заболевание может осложниться ТТП. Приведено клиническое наблюдение.

   Заключение

   Заключение. При сочетании тромбоцитопении с гемолизом и при отсутствии полиорганной патологии без исследования плазменной активности ADAMTS113 сложно дифференцировать ИТП и ТТП. При сочетании ИТП и ТТП у одного и того же больного исследование плазменной активности ADAMTS13 позволяет выявить ведущий механизмтромбоцитопении и выбрать тактику лечения.

   Introduction

   Introduction. When establishing a diagnosis of thrombotic thrombocytopenic purpura (TTP) for the first time, other diseases occurring with thrombocytopenia may be misdiagnosed.

   Aim

   Aim: to analyze diagnostic difficulties and errors of TTP.

   Materials and methods

   Materials and methods. In total, 54 patients (44 women and 10 men) aged 18 to 83 years (median 33 years), who were diagnosed TTP from 2019 to 2023, were included in the study. Anamnesis, baseline hemoglobin, platelets, erythrocytes, lactate dehydrogenase and bilirubin serum concentrations, pre-established diagnoses, treatment, and outcomes were analyzed.

   Results

   Results. Initially TTP was suspected only in 19 (35 %) patients. In 12 out of 54 patients immune thrombocytopenia (ITP) was initially misdiagnosed. Fisher-Evans syndrome was initially suspected in 3 patients. In sporadic cases, myelodysplastic syndrome, systemic lupus erythematosus / antiphospholipid syndrome, autoimmune hemolytic anemia were initially suspected. Among 15 women with associated with pregnancy TTP in 8 (53.3 %), HELLP syndrome was initially misdiagnosed. In total, 12 (22.2 %) of 54 TTP patients died. In 11/12 non-survival patients, TTP treatment was never started, because the doctors were waiting for the ADAMTS13 test results. A case report of Sequential Immune Thrombocytopenia (ITP) and TTP in the same Patient is described.

   Conclusion

   Conclusion. When thrombocytopenia is combined with hemolysis and in the absence of multiple organ pathology, it is difficult to differentiate ITP and TTP without examining the plasma activity of ADAMTS113. With a combination of ITP and TTP in the same patient, the study of ADAMTS13 plasma activity makes it possible to identify the leading mechanism of thrombocytopenia and choose treatment tactics.

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