bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2021-66-2-280-311bloodjour-284Research ArticleКЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИCLINICAL RECOMMENDATIONSНациональные клинические рекомендации по диагностике и лечению мастоцитозаNational clinical recommendations for the diagnosis and treatment of mastocytosishttps://orcid.org/0000-0002-2119-3775МеликянА. Л.MelikyanA. L.

Меликян Анаит Левоновна, доктор медицинских наук, заведующая отделением стандартизации методов лечения

125167, Москва

Anait L. Melikyan, Dr. Sci. (Med.), Head of the Department of Standardisation of Treatment Methods

125167, Moscow

anoblood@mail.ruhttps://orcid.org/0000-0001-9045-8653СуборцеваИ. Н.SubortsevaI. N.

Суборцева Ирина Николаевна, кандидат медицинских наук, старший научный сотрудник отделения стандартизации методов лечения

125167, Москва

Irina N. Subortseva, Cand. Sci. (Med.), Senior Researcher, Department of Standardisation of Treatment Methods

125167, Moscow

soubortseva@yandex.ruhttps://orcid.org/0000-0003-3536-0770ШуваевВ. А.ShuvaevV. A.

Шуваев Василий Анатольевич, кандидат медицинских наук, ведущий научный сотрудник, ФГБУ «Российский научно-исследовательский институт гематологии и трансфузиологии Федерального медико-биологического агентства России», руководитель гематологического центра ГКБ им. В.В. Вересаева Департамента здравоохранения г. Москвы»

191024, Санкт-Петербург

Vasily A. Shuvaev, Cand. Sci. (Med.), Leading Researcher, Russian Research Institute of Hematology and Transfusiology; Head of the Hematology Center, Veresaev City Clinical Hospital

191024, St. Petersburg

shuvaev77@mail.ruhttps://orcid.org/0000-0003-0752-0757МорозоваЕ. В.MorozovaE. V.

Морозова Елена Владиславовна, кандидат медицинских наук, руководитель отдела онкологии, гематологии и трансплантологии для взрослых, НИИ детской онкологии, гематологии и трансплантологии им. Р.М.  Горбачевой ФГБОУ ВО «Санкт-Петербургский государственный медицинский университет им. акад. И.П.  Павлова» Министерства здравоохранения Российской Федерации

197022, Санкт-Петербург

Elena V. Morozova, Cand. Sci. (Med.), Head of the Department of Oncology, Hematology and Transplantation in Adults, Raisa Gorbacheva Memorial Research Institute for Pediatric Oncology, Hematology and Transplantation; Pavlov First Saint-Petersburg State Medical University

197022, St. Petersburg

dr_morozova@mail.ruhttps://orcid.org/0000-0002-6757-985XЛукинаК. А.LukinaK. A.

Лукина Кира Анатольевна, кандидат медицинских наук, заведующая отделом организации и оказания медицинской помощи с  применением телемедицинских технологий по профилю «гематология»

125167, Москва

Kira A. Lukina, Cand. Sci. (Med.), Head of the Department of Telemedicine Technologies for Hematological Aid

125167, Moscow

kira1404@gmail.comhttps://orcid.org/0000-0002-2793-8862АртемьеваС. И.ArtemievaS. I.

Артемьева Софья Иосифовна, младший научный сотрудник отдела клинической дерматовенерологии и косметологии

119071, Москва

Sof’ya I. Artemieva, Junior Researcher, Department of Clinical Dermatovenereology and Cosmetology

119071, Moscow

sofya.chern@gmail.comhttps://orcid.org/0000-0002-3875-4030ЛьвовА. Н.LivovA. N.

Львов Андрей Николаевич, доктор медицинских наук, профессор, руководитель отдела клинической дерматовенерологии и косметологии

119071, Москва

Andrey N. Livov, Dr. Sci. (Med.), Professor, Head of the Department of Clinical Dermatovenereology and Cosmetology

119071, Moscow

alvov@mail.ruhttps://orcid.org/0000-0002-9191-5091БайковВ. В.BaykovV. V.

Байков Вадим Валентинович, доктор медицинских наук, профессор, заведующий лабораторией патоморфологии

197022, Санкт-Петербург

Vadim V. Baykov, Dr. Sci. (Med.), Professor, Head of the Laboratory of Morbid Morphology

197022, St. Petersburg

baikov02@mail.ruhttps://orcid.org/0000-0002-1116-7880ВиноградоваО. Ю.VinogradovaO. Yu.

Виноградова Ольга Юрьевна, доктор медицинских наук, заведующая, Московский городской гематологический центр ГБУЗ «Городская клиническая больница имени С.П. Боткина Департамента здравоохранения города Москвы»; профессор кафедры онкологии, гематологии и лучевой терапии, ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И.  Пирогова» Министерства здравоохранения Российской Федерации

125284, Москва

Olga Yu. Vinogradova, Dr. Sci. (Med.), Head of the Botkin Hospital Moscow Hematology Center; Prof., Chair of Oncology, Hematology and Radiation Therapy, Pirogov Russian National Research Medical University

125284, Moscow

olgavinz@mail.ruhttps://orcid.org/0000-0001-8818-8949ГалстянГ. М.GalstyanG. M.

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

125167, Москва

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

125167, Moscow

gengalst@gmail.comhttps://orcid.org/0000-0002-3883-185XГилязитдиноваЕ. А.GilyazitdinovaE. A.

Гилязитдинова Елена Александровна, врач-гематолог отделения стандартизации методов лечения

125167, Москва

Elena A. Gilyazitdinova, Physician (haematology), Department of Standardisation of Treatment Methods

125167, Moscow

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

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

125167, Москва

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

125167, Moscow

dvirnyk.v@blood.ruhttps://orcid.org/0000-0001-5723-6573ЖуковаО. В.ZhukovaO. V.

Жукова Ольга Валентиновна, доктор медицинских наук, профессор, заведующий кафедрой кожных и венерических болезней медицинского института РУДН; главный врач ГБУЗ «Московский научно-практический Центр дерматовенерологии и косметологии Департамента здравоохранения города Москвы»

119071, Москва

Olga V. Zhukova, Dr. Sci. (Med.), Professor, Head of the Chair of Skin and Venereal Diseases, Institute of Medicine, RUDN University; Chief Physician

119071, Moscow

klinderma@inbox.ruhttps://orcid.org/0000-0002-1082-8659КовригинаА. М.KovriginaA. M.

Ковригина Алла Михайловна, доктор биологических наук, заведующая отделением патологической анатомии

125167, Москва

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

125167, Moscow

kovrigina.alla@gmail.comhttps://orcid.org/0000-0003-0246-2906КохноА. В.KohnoA. V.

Кохно Алина Владимировна, кандидат медицинских наук, начальник клинико-диагностического отдела

125167, Москва

Alina V. Kohno, Cand. Sci. (Med.), Head of Clinical and Diagnostic Department

125167, Moscow

anilako@rambler.ruhttps://orcid.org/0000-0001-6201-6276КузьминаЛ. А.KuzminaL. A.

Кузьмина Лариса Анатольевна, кандидат медицинских наук, заведующая отделением интенсивной высокодозной химиотерапии и трансплантации костного мозга

125167, Москва

Larisa A. Kuzmina, Cand. Sci. (Med.), Head of the Department of Intensive High-Dose Chemotherapy and Bone Marrow Transplantation

125167, Moscow

kuzlara@rambler.ruhttps://orcid.org/0000-0003-3290-7961ЛомаиаЕ. Г.LomaiaE. G.

Ломаиа Елза Галактионовна, кандидат медицинских наук, ведущий научный сотрудник научно-исследовательского отдела клинической онкологии Института онкологии и гематологии

197341, Санкт-Петербург

Elza G. Lomaia, Cand. Sci. (Med.), Leading Researcher, Research Department of Clinical Oncology, Institute of Oncology and Hematology

197341, St. Petersburg

lomelza@gmail.comhttps://orcid.org/0000-0003-1437-3337ЛукинаЕ. 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/0000-0001-5958-0490МартынкевичИ. С.MartynkevichI. S.

Мартынкевич Ирина Степановна, доктор биологических наук, руководитель лаборатории молекулярной генетики

191024, Санкт-Петербург

Irina S. Martynkevich, Dr. Sci. (Biol.), Head of the Laboratory of Molecular Genetics

191024, St. Petersburg

mis2907@mail.ruhttps://orcid.org/0000-0001-7493-0030МитинаТ. А.MitinaT. A.

Митина Татьяна Алексеевна, доктор медицинских наук, ведущий научный сотрудник, руководитель отделения клинической гематологии и иммунотерапии

125284, Москва

Тatiana A. Mitina, Dr. Sci. (Med.), Leading Researcher, Head of the Department of Clinical Hematology and Immunotherapy, Vladimirsky Moscow Regional Research and Clinical Institute

125284, Moscow

mi_69@inbox.ruhttps://orcid.org/0000-0002-9578-5490ПотекаевН. Н.PotekaevN. N.

Потекаев Николай Николаевич, доктор медицинских наук, профессор, заведующий кафедрой кожных болезней и косметологии, ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И.  Пирогова» Министерства здравоохранения Российской Федерации; директор, ГБУЗ «Московский научно-практический Центр дерматовенерологии и косметологии Департамента здравоохранения города Москвы»

119071, Москва,

117997, Москва

Nikolay N. Potekaev, Dr. Sci. (Med.), Professor, Head of the Chair of Skin Diseases and Cosmetology, Pirogov Russian National Research Medical University; Director, Moscow Scientifi c and Practical Center of Dermatovenereology and Сosmetology

119071, Moscow,

117997, Moscow

klinderma@mail.ruhttps://orcid.org/0000-0001-9463-9187СудариковА. Б.SudarikovA. B.

Судариков Андрей Борисович, доктор биологических наук, заведующий лабораторией молекулярной гематологии

125167, Москва

Andrey B. Sudarikov, Dr. Sci. (Biol.), Head of the Laboratory of Molecular Hematology

125167, Moscow

dusha@blood.ruhttps://orcid.org/0000-0002-0238-6563ШатохинаЕ. А.ShatokhinaE. A.

Шатохина Евгения Афанасьевна, кандидат медицинских наук, доцент кафедры дерматовенерологии и косметологии, ФГБУ ДПО «Центральная государственная медицинская академия» Управления делами Президента Российской Федерации; дерматовенеролог клинического отдела, ФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской Федерации

125167, Москва,

121359, Москва

Evgenia A. Shatokhina, Cand. Sci. (Med.), Associate Professor, Chair of Dermatovenerology and Cosmetology, Central State Medical Academy of the Russian President Administration; Physician (dermatovenerology), Clinical Department, National Research Center for Hematology

125167, Moscow

121359, Moscow

e.a.shatokhina@gmail.comhttps://orcid.org/0000-0001-6231-971XФригоН. В.FrigoN. V.

Фриго Наталия Владиславовна, доктор медицинских наук, заместитель директора по  науке

119071, Москва

Natalia V. Frigo, Dr. Sci. (Med.), Deputy Director for Science

119071, Moscow

frigo2013@yandex.ruhttps://orcid.org/0000-0003-1393-856XСавченкоВ. Г.SavchenkoV. G.

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

125167, Москва

Valery G. Savchenko, Dr. Sci. (Med.), Professor, Full Member of the Russian Academy of Sciences, Director

125167, Moscow

director@blood.ruФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Research Center for HematologyRussian FederationФГБУ «Российский научно-исследовательский институт гематологии и трансфузиологии Федерального медико-биологического агентства России»РоссияRussian Research Institute of Hematology and TransfusiologyRussian FederationНИИ детской онкологии, гематологии и трансплантологии им. Р.М. Горбачевой ФГБОУ ВО «Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова» Министерства здравоохранения Российской ФедерацииРоссияRaisa Gorbacheva Memorial Research Institute for Pediatric Oncology, Hematology and Transplantation, Pavlov First Saint-Petersburg State Medical UniversityRussian FederationГБУЗ «Московский научно-практический Центр дерматовенерологии и косметологии Департамента здравоохранения города Москвы»РоссияMoscow Scientific and Practical Center of Dermatovenereology and СosmetologyRussian FederationГБУЗ «Московский научно-практический Центр дерматовенерологии и косметологии Департамента здравоохранения города Москвы»РоссияMoscow Scientific and Practical Center of Dermatovenereology and Сosmetology;Russian FederationМосковский городской гематологический центр ГБУЗ «Городская клиническая больница имени С.П. Боткина Департамента здравоохранения города Москвы»РоссияBotkin Hospital Moscow Hematology CenterRussian FederationФГБУ «Национальный медицинский исследовательский центр им. В.А. Алмазова» Министерства здравоохранения Российской ФедерацииРоссияAlmazov National Medical Research CentreRussian FederationГБУЗ «Московский научно-практический Центр дерматовенерологии и косметологии Департамента здравоохранения города Москвы»; ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Министерства здравоохранения Российской ФедерацииРоссияMoscow Scientific and Practical Center of Dermatovenereology and Сosmetology; Pirogov Russian National Research Medical UniversityRussian FederationФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской Федерации; ФГБУ ДПО «Центральная государственная медицинская академия» Управления делами Президента Российской ФедерацииРоссияNational Research Center for Hematology; Central State Medical Academy of the Russian President AdministrationRussian Federation202101092021662280311Copyright © Меликян А.Л., Суборцева И.Н., Шуваев В.А., Морозова Е.В., Лукина К.А., Артемьева С.И., Львов А.Н., Байков В.В., Виноградова О.Ю., Галстян Г.М., Гилязитдинова Е.А., Двирнык В.Н., Жукова О.В., Ковригина А.М., Кохно А.В., Кузьмина Л.А., Ломаиа Е.Г., Лукина Е.А., Мартынкевич И.С., Митина Т.А., Потекаев Н.Н., Судариков А.Б., Шатохина Е.А., Фриго Н.В., Савченко В.Г., 20212021Меликян А.Л., Суборцева И.Н., Шуваев В.А., Морозова Е.В., Лукина К.А., Артемьева С.И., Львов А.Н., Байков В.В., Виноградова О.Ю., Галстян Г.М., Гилязитдинова Е.А., Двирнык В.Н., Жукова О.В., Ковригина А.М., Кохно А.В., Кузьмина Л.А., Ломаиа Е.Г., Лукина Е.А., Мартынкевич И.С., Митина Т.А., Потекаев Н.Н., Судариков А.Б., Шатохина Е.А., Фриго Н.В., Савченко В.Г.Melikyan A.L., Subortseva I.N., Shuvaev V.A., Morozova E.V., Lukina K.A., Artemieva S.I., Livov A.N., Baykov V.V., Vinogradova O.Y., Galstyan G.M., Gilyazitdinova E.A., Dvirnyk V.N., Zhukova O.V., Kovrigina A.M., Kohno A.V., Kuzmina L.A., Lomaia E.G., Lukina E.A., Martynkevich I.S., Mitina T.A., Potekaev N.N., Sudarikov A.B., Shatokhina E.A., Frigo N.V., Savchenko V.G.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/284Введение

Введение. Рекомендации посвящены современному состоянию проблемы диагностики и лечения мастоцитоза.

Цель — предоставить консолидированное мнение российских экспертов по лечению взрослых больных мастоцитозом.

Основные сведения

Основные сведения. Разработаны рекомендации с  учетом зарубежных публикаций и  отечественного опыта, на  основе мировых клинических исследований по  лечению системного мастоцитоза, кожного мастоцитоза, тучноклеточного лейкоза и других форм мастоцитоза. Показано значение молекулярно-генетического исследования костного мозга и  периферической крови на  наличие перестроек гена KITD816V. Приведены схемы лечения с применением мидостаурина, иматиниба, кладрибина, гидроксикарбамида, интерферон альфа, трансплантации гемопоэтических стволовых клеток. Приведен прогноз при различных формах мастоцитоза.

Introduction

Introduction. Recommendations cover the current state of diagnosis and treatment of mastocytosis.

Aim — а consolidation of the Russian experts’ opinion on treatment for adult mastocytosis.

Main findings

Main findings. The recommendations have been developed taking into account foreign literature, national experience and world clinical evidence on therapy for systemic and cutaneous mastocytoses, mast cell leukaemia and other mastocytosis forms. The significance of bone marrow and peripheral blood molecular genetic testing for the presence of KITD816V gene variants is demonstrated. The treatment regimens described are based on midostaurin, imatinib, cladribine, hydroxycarbamide, interferon alfa and haematopoietic stem cell transplantation. Prognosis in different forms of mastocytosis is provided.

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