bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2022-68-4-530-555bloodjour-493Research ArticleРЕКОМЕНДАЦИИRЕCOMMENDATIONSРекомендации по диагностике и лечению идиопатической тромбоцитопенической пурпурыGuidelines for the diagnosis and management of idiopathic thrombocytopenic purpurahttps://orcid.org/0000-0002-2119-3775МеликянА. Л.MelikyanA. L.

Меликян Анаит Левоновна, доктор медицинских наук, руководитель научно-клинического отделения стандартизации методов лечения

125167

г. Москва

Anahit L. Melikyan, Dr. Sci. (Med.), Head of the Department of Standardization of Treatment Methods

125167

Moscow

anoblood@mail.ruhttps://orcid.org/0000-0002-1099-8092ПустоваяЕ. И.PustovayaE. I.

Пустовая Елена Игоревна, кандидат медицинских наук, старший научный сотрудник, гематолог научно-клинического отделения стандартизации методов лечения

125167

г. Москва

Elena I. Pustovaya, Can. Sci. (Med.), hematologist, Department for the Standartization of treatment methods

125167

Moscow

elenapustov@mail.ruЦветаеваН. В.TsvetaevaN. V.

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

125167

г. Москва

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

125167

Moscow

tsvetaeva51@gmail.comhttps://orcid.org/0000-0001-6038-9474ЕгороваЕ. К.EgorovaE. K.

Егорова Елена Константиновна, кандидат медицинских наук, гематолог научно-клинического отделения стандартизации методов лечения

125167

г. Москва

Elena K. Egorova, Can. Sci. (Med.), hematologist, Department for the Standartization of treatment methods

125167

Moscow

gorova.e@blood.ruhttps://orcid.org/0000-0003-3662-9751ДанишянК. И.DanishyanK. I.

Данишян Карен Исмаилович, доктор медицинских наук, заведующий отделением хирургии

125167

г. Москва

Karen I. Danishyan, Dr. Sci. (Med.), Head of the Department of Surgery

125167

Moscow

ntanisian@gmail.comhttps://orcid.org/0000-0001-8818-8949ГалстянГ. М.GalstyanG. M.

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

125167

г. Москва

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

125167

Moscow

gengalst@gmail.comФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for HematologyRussian Federation202328122023684530555Copyright © Меликян А.Л., Пустовая Е.И., Цветаева Н.В., Егорова Е.К., Данишян К.И., Галстян Г.М., 20232023Меликян А.Л., Пустовая Е.И., Цветаева Н.В., Егорова Е.К., Данишян К.И., Галстян Г.М.Melikyan A.L., Pustovaya E.I., Tsvetaeva N.V., Egorova E.K., Danishyan K.I., Galstyan G.M.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/493

Введение. Первичная иммунная тромбоцитопения (ИТП) — это аутоиммунное заболевание, обусловленное выработкой антител к структурам мембраны тромбоцитов и их предшественников — мегакариоцитов, что вызывает не только повышенную деструкцию тромбоцитов, но и неадекватный тромбоцитопоэз, характеризующийся изолированной тромбоцитопенией ниже 100,0×109/л и наличием/отсутствием геморрагического синдрома различной степени выраженности.Цель — представить современные рекомендации по диагностике и лечению ИТП.Основные сведения. Рекомендации разработаны на основе опыта российских и международных экспертов, руководства международной рабочей группы по изучению первичной ИТП, рекомендаций европейского и американского обществ гематологов с использованием новых данных о развитии и течении ИТП. Представлены подходы к терапии заболевания с использованием новых лекарственных препаратов из группы агонистов рецепторов тромбопоэтина

Introduction. Primary immune thrombocytopenia (ITP) is an autoimmune disease caused by the production of antibodies to the membrane structures of platelets and their precursors — megakaryocytes, which causes not only increased destruction of platelets, but also inadequate thrombocytopoiesis, characterized by isolated thrombocytopenia below 100.0×109/L and the presence/absence of hemorrhagic syndrome of varying severity.Aim: to present modern recommendations for the diagnosis and treatment of ITPBasic information. The recommendations are based on the experience of Russian and international experts, the leadership of the international working group on the study of primary immune thrombocytopenia, and recommendations of the European and American Societies of Hematology using new data on the development and course of ITP. Approaches to the treatment of the disease using new drugs from the group of thrombopoietin receptor agonists are present

идиопатическая тромбоцитопеническая пурпурапервичная и вторичная иммунная тромбоцитопениядиагностика ИТПтерапия 1-й2-й и 3-й линийбеременностьthrombocytopenic purpuraprimary and secondary immune thrombocytopeniadiagnosis of ITPtherapy of the 1st2nd and 3rd linespregnancyReferencesRodeghiero F., Stasi R., Gernsheimer T., et al. Standardization of terminology, defi nitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from international working group. Blood. 2009; 113(11): 2386–93. DOI: 10.1182/blood-2008-07-162503.Rodeghiero F., Stasi R., Gernsheimer T., et al. Standardization of terminology, defi nitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from international working group. Blood. 2009; 113(11): 2386–93. DOI: 10.1182/blood-2008-07-162503.Cines D.B., Cuker A., Semple J.W. Pathogenesis of immune thrombocytopenia. Presse Med. 2014; 43(4 Pt2): e49–59. 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The authors declare that there are no conflicts of interest present.