bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2022-67-3-426-439bloodjour-390Research ArticleКЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИCLINICAL RECOMMENDATIONSКлинические рекомендации по ведению больных с пароксизмальной ночной гемоглобинуриейClinical guidelines for the management of patients with paroxysmal nocturnal hemoglobinuriahttps://orcid.org/0000-0001-8188-5557СавченкоВ. Г.SavchenkoV. G.

Савченко Валерий Григорьевич , доктор медицинских наук, профессор, академик РАН, генеральный директор

125167, Москва

Valery G. Savchenko , Dr. Sci. (Med.), Professor, Academician of RAS, CEO

125167, Moscow

svg@blood.ruhttps://orcid.org/0000-0002-8774-850XЛукинаЕ. А.LukinaE. A.

Лукина Елена Алексеевна, доктор медицинских наук, профессор, заведующая отделением орфанных заболеваний

125167, Москва

Elena A. Lukina, Dr. Sci. (Med.), Professor, Head of the Department of Orphan Diseases

125167, Moscow

elenalukina02@gmail.comhttps://orcid.org/0000-0002-2449-2682МихайловаЕ. А.MikhaylovaE. A.

Михайлова Елена Алексеевна, доктор медицинских наук, профессор, ведущий научный сотрудник отделения интенсивной высокодозной химиотерапии гемобластозов и депрессий кроветворения

125167, Москва

Elena A. Mikhaylova, Dr. Sci. (Med.), Professor, Leading Researcher of the Department of Intensive High-Dose Chemotherapy for Hemoblastosis and Hematopoietic Depressions

125167, Moscow

mikhaylova.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, Department of Orphan Diseases

125167, Moscow

tsvetaeva.n@blood.ruhttps://orcid.org/0000-0003-0643-8807ЛатышевВ. Д.LatyshevV. D.

Латышев Виталий Дмитриевич, гематолог отделения орфанных заболеваний

125167, Москва

Vitalii D. Latyshev, Hematologist, Department of Orphan Diseases

125167, Moscow

latyshevVD@gmail.comhttps://orcid.org/0000-0002-6757-985XЛукинаК. А.LukinaK. A.

Лукина Кира Анатольевна, кандидат медицинских наук, гематолог отделения орфанных заболеваний

125167, Москва

Kira A. Lukina, Cand. Sci. (Med.), Hematologist, Department of Orphan Diseases

125167, Moscow

kira1404@gmail.comhttps://orcid.org/0000-0003-0934-6094ФидароваЗ. Т.FidarovaZ. T.

Фидарова Залина Таймуразовна, кандидат медицинских наук, заведующая отделением гемобластозов и депрессий кроветворения

125167, Москва

Zalina T. Fidarova, Cand. Sci. (Med.), Head of the Department of Hemoblastosis and Hematopoietic Depressions

125167, Moscow

https://orcid.org/0000-0002-8490-6066ГальцеваИ. В.GaltsevaI. V.

Гальцева Ирина Владимировна, кандидат медицинских наук, заведующая лабораторией иммунофенотипирования клеток крови и костного мозга

125167, Москва

Irina V. Galtseva, Cand. Sci. (Med.), Head of the Laboratory for Immunophenotyping of Blood Cells and Bone Marrow

125167, Moscow

irinagaltseva@gmail.comhttps://orcid.org/0000-0002-9877-0796ДвирныкВ. Н.DvirnikV. N.

Двирнык Валентина Николаевна, кандидат медицинских наук, заведующая централизованной клинико-диагностической лабораторией

125167, Москва

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

125167, Moscow

dvirnyk.v@blood.ruhttps://orcid.org/0000-0002-9368-6050ПтушкинВ. В.PtushkinV. V.

Птушкин Вадим Вадимович, доктор медицинских наук, главный внештатный специалист-гематолог ДЗ г. Москвы, заместитель главного врача по гематологии

125284, Москва

Vadim V. Ptushkin, Dr. Sci. (Med.), Chief Hematology Specialist of the Moscow Health Department, Deputy Chief Physician for Hematology

125284, Moscow

vadimvadim@inbox.ruhttps://orcid.org/0000-0002-1235-4530АфанасьевБ. В.AfanasyevB. V.

Афанасьев Борис Владимирович, доктор медицинских наук, профессор, директор

197022, Санкт-Петербург

Boris V. Afanasyev, Dr. Sci. (Med.), Professor, Director

197022, Saint-Petersburg

bmt-director@1spbgmu.ruhttps://orcid.org/0000-0002-9589-4136КулагинА. Д.KulaginA. D.

Кулагин Александр Дмитриевич, доктор медицинских наук, директор

197022, Санкт-Петербург

Alexander D. Kulagin, Dr. Sci. (Med.), Director

197022, Saint-Petersburg

bmt-director@1spbgmu.ruhttps://orcid.org/0000-0002-9253-6181ШиловаЕ. Р.ShilovaE. R.

Шилова Елена Романовна, кандидат медицинских наук, врач-гематолог

191024, Санкт-Петербург

Elena R. Shilova, Cand. Sci. (Med.), Hematologist

191024, Saint-Petersburg

sherom08@inbox.ruhttps://orcid.org/0000-0002-0016-6698МасчанА. А.MaschanA. A.

Масчан Алексей Александрович, доктор медицинских наук, профессор, член-корреспондент РАН, заместитель генерального директора, директор Института гематологии, иммунологии и клеточных технологий

117997, Москва

Aleksei A. Maschan, Dr. Sci. (Med.), Professor, Corresponding Member of RAS, Deputy General Director, Director of the Institute of Hematology, Immunology and Cell Technologies

117997, Moscow

amaschan@mail.ruСметанинаН. С.SmetaninaN. S.

Сметанина Наталья Сергеевна, доктор медицинских наук, профессор, заместитель директора Института гематологии, иммунологии и клеточных технологий

117997, Москва

Nataliya S. Smetanina, Dr. Sci. (Med.), Professor, Deputy Director of the Institute of Hematology, Immunology and Cell Technologies

117997, Moscow

nataliya.smetanina@fnkc.ruhttps://orcid.org/0000-0002-6405-3422ЛуговскаяС. А.LugovskayaS. A.

Луговская Светлана Алексеевна, доктор медицинских наук, профессор кафедры клинической лабораторной диагностики

127994, Москва

Svetlana A. Lugovskaya, Dr. Sci. (Med.), Professor of the Department of Clinical Laboratory Diagnostics

127994, Moscow

slugovskaya@mail.ruФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for HematologyRussian FederationГБУЗ «Городская клиническая больница имени С.П. Боткина» Департамента здравоохранения г. МосквыРоссияBotkin City Clinical Hospital of the Moscow Health DepartmentRussian FederationНИИ детской онкологии, гематологии и трансплантологии им. Р.М. Горбачевой, ФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет им акад. И.П. Павлова» Министерства здравоохранения Российской ФедерацииРоссияRaisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, Pavlov First State Medical University of St. PetersburgRussian FederationНИИ детской онкологии, гематологии и трансплантологии им. Р.М. Горбачевой, ФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова» Министерства здравоохранения Российской ФедерацииРоссияRaisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, Pavlov First State Medical University of St. PetersburgRussian FederationФГБУ «Российский научно-исследовательский институт гематологии и трансфузиологии Федерального медико-биологического агентства»РоссияRussian Research Institute of Hematology and Transfusiology of the Federal Medical and Biological AgencyRussian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии имени Дмитрия Рогачева» Министерства здравоохранения Российской ФедерацииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and ImmunologyRussian FederationФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Министерства здравоохранения Российской ФедерацииРоссияRussian Medical Academy of Continuing Professional EducationRussian Federation202223102022673426439Copyright © Савченко В.Г., Лукина Е.А., Михайлова Е.А., Цветаева Н.В., Латышев В.Д., Лукина К.А., Фидарова З.Т., Гальцева И.В., Двирнык В.Н., Птушкин В.В., Афанасьев Б.В., Кулагин А.Д., Шилова Е.Р., Масчан А.А., Сметанина Н.С., Луговская С.А., 20222022Савченко В.Г., Лукина Е.А., Михайлова Е.А., Цветаева Н.В., Латышев В.Д., Лукина К.А., Фидарова З.Т., Гальцева И.В., Двирнык В.Н., Птушкин В.В., Афанасьев Б.В., Кулагин А.Д., Шилова Е.Р., Масчан А.А., Сметанина Н.С., Луговская С.А.Savchenko V.G., Lukina E.A., Mikhaylova E.A., Tsvetaeva N.V., Latyshev V.D., Lukina K.A., Fidarova Z.T., Galtseva I.V., Dvirnik V.N., Ptushkin V.V., Afanasyev B.V., Kulagin A.D., Shilova E.R., Maschan A.A., Smetanina N.S., Lugovskaya S.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.htjournal.ru/jour/article/view/390Введение

Введение. Пароксизмальная ночная гемоглобинурия (ПНГ) — это редкое приобретенное клональное заболевание системы крови, характеризующееся внутрисосудистым гемолизом, дисфункцией костного мозга и повышенным риском тромботических и органных осложнений.

Цель — предоставить актуальные клинические рекомендации по оказанию медицинской помощи взрослым и детям с ПНГ.

Основные сведения

Основные сведения. Экспертами ассоциации содействия развитию гематологии, трансфузиологии и трансплантации костного мозга «Национальное гематологическое общество» и общественной организации «Национальное общество детских гематологов и онкологов» разработаны актуальные клинические рекомендации по оказанию медицинской помощи взрослым и детям с ПНГ. В рекомендациях подробно рассмотрены вопросы этиологии, патогенеза, эпидемиологии и клинических проявлений заболевания. Особое внимание уделено диагностике, дифференциальной диагностике и лечению ПНГ, основанным на принципах доказательности.

Introduction

Introduction. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal disease of the blood system characterized by intravascular hemolysis, bone marrow dysfunction and an increased risk of thrombotic and organ complications.

Aim — to provide relevant clinical recommendations for the provision of medical care to adults and children with PNH.

Basic information

Basic information. Experts from the National Hematological Society association which is focused on the promotion of hematology, transfusiology and bone marrow transplantation along with experts from the public organization, National Society of Pediatric Hematologists and Oncologists, have developed current clinical recommendations for providing medical care to adults and children with PNH. The recommendations address in detail the issues of etiology, pathogenesis, epidemiology, and clinical manifestations of the disease. Special attention is paid to the diagnosis, differential diagnosis, and treatment of PNH based on the principles of evidence.

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