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<article article-type="research-article" dtd-version="1.3" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">bloodjour</journal-id><journal-title-group><journal-title xml:lang="ru">Гематология и трансфузиология</journal-title><trans-title-group xml:lang="en"><trans-title>Russian journal of hematology and transfusiology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">0234-5730</issn><issn pub-type="epub">2411-3042</issn><publisher><publisher-name>ООО Издательский дом «Практика»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.35754/0234-5730-2023-68-4-432-442</article-id><article-id custom-type="elpub" pub-id-type="custom">bloodjour-485</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОРИГИНАЛЬНЫЕ СТАТЬИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL ARTICLES</subject></subj-group></article-categories><title-group><article-title>Протокол «АККЛ НИИ ДОиГ 2003»: результаты лечения анапластической крупноклеточной лимфомы у детей в рамках сравнительного многоцентрового клинического исследования</article-title><trans-title-group xml:lang="en"><trans-title>ALCL Protocol of the DOIG Research Institute 2003: results of treatment of anaplastic large cell lymphoma in children in a comparative multicenter clinical trial</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1709-0498</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Волкова</surname><given-names>А. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Volkova</surname><given-names>A. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Волкова Анастасия Сергеевна, детский онколог отделения детской онкологии и  гематологии (химиотерапии гемобластозов) № 1</p><p>115478</p><p>Москва</p></bio><bio xml:lang="en"><p>Anastasiya S. Volkova, Pediatric Oncologist of the Department of Pediatric Oncology and Hematology (Chemotherapy of Hemoblastosis) No. 1</p><p>115478</p><p>Moscow</p></bio><email xlink:type="simple">anastasia.sergeevna.volkova@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1469-2365</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Валиев</surname><given-names>Т. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Valiev</surname><given-names>T. T.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Валиев Тимур Теймуразович, доктор медицинских наук, заведующий отделением детской онкологии и  гематологии (химиотерапии гемобластозов) № 1</p><p>115478</p><p>Москва</p></bio><bio xml:lang="en"><p>Timur T. Valiev, Dr. Sci. (Med.), Head of the Department of Pediatric Oncology and Hematology (Chemotherapy of Hemoblastosis) No. 1</p><p>115478</p><p>Moscow</p></bio><email xlink:type="simple">timurvaliev@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Батманова</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Batmanova</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Батманова Наталья Андреевна, кандидат медицинских наук, детский онколог отделения детской онкологии и гематологии (химиотерапии гемобластозов) № 1</p><p>115478</p><p>Москва</p></bio><bio xml:lang="en"><p>Natalia A. Batmanova, Cand. Sci. (Med.), Pediatric Oncologist of the Department of Pediatric Oncology and Hematology (Chemotherapy of Hemoblastosis) No. 1</p><p>115478</p><p>Moscow</p></bio><email xlink:type="simple">batmanova_nataly@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2614-2560</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Юлдашева</surname><given-names>С. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Yuldasheva</surname><given-names>S. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Юлдашева Светлана Александровна, заведующая отделением детской онкологии</p><p>283045</p><p>Донецк</p></bio><bio xml:lang="en"><p>Svetlana N. Yuldasheva, Head of the Pediatric Oncology Department</p><p>283045Donetsk</p></bio><email xlink:type="simple">oogdinvh@mail.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2116-2087</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гордеева</surname><given-names>З. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Gordeeva</surname><given-names>Z. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Гордеева Зинаида Семеновна, детский онколог, заместитель главного врача по медицинской части</p><p>424004</p><p>Йошкар-Ола</p></bio><bio xml:lang="en"><p>Zinaida S. Gordeeva, Pediatric Oncologist, Deputy Chief Medical Officer</p><p>424004</p><p>Ioshkar-Ola</p></bio><email xlink:type="simple">dgb@mari-el.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0002-0269-8011</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сабанцев</surname><given-names>С. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Sabantsev</surname><given-names>S. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Сабанцев Степан Леонидович, гематолог, детский онколог, заведующий детским онкогематологическим отделением</p><p>424004</p><p>Йошкар-Ола</p></bio><bio xml:lang="en"><p>Stepan L. Sabantsev, Hematologist, Pediatric Oncologist, Head of the Pediatric Oncohematology Department</p><p>424004</p><p>Ioshkar-Ola</p></bio><email xlink:type="simple">sabantsevdgb@yandex.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0009-0002-0269-8011</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Алексерова</surname><given-names>Г. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Aleskerova</surname><given-names>G. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Сабанцев Степан Леонидович, гематолог, детский онколог, заведующий детским онкогематологическим отделением</p><p>AZ1012</p><p>Баку</p></bio><bio xml:lang="en"><p>Gunel A. Aleskerova, Cand. Sci. (Med.), Head of the Pediatric Chemotherapy Department</p><p>AZ1012</p><p>Baku</p></bio><email xlink:type="simple">sabantsevdgb@yandex.ru</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-9833-5156</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Осипова</surname><given-names>И. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Osipova</surname><given-names>I. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Осипова Ильсия Вагизовна, главный внештатный детский онколог-гематолог</p><p>420138</p><p>Казань</p></bio><bio xml:lang="en"><p>Ilsia V. Osipova, Chief Freelance Specialist in Pediatric Oncology</p><p>420138</p><p>Kazan</p></bio><email xlink:type="simple">ivos29@mail.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6131-1783</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Варфоломеева</surname><given-names>С. Р.</given-names></name><name name-style="western" xml:lang="en"><surname>Varfolomeeva</surname><given-names>S. R.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Варфоломеева Светлана Рафаэлевна, доктор медицинских наук, профессор, директор</p><p>115478</p><p>Москва</p></bio><bio xml:lang="en"><p>Svetlana R. Varfolomeeva, Dr. Sci. (Med.), Professor, Director of the Research Institute of Pediatric Oncology and Hematology</p><p>115478</p><p>Moscow</p></bio><email xlink:type="simple">s.varfolomeeva@ronc.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>НИИ детской онкологии и гематологии ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Blokhin National Medical Research Centre of Oncology of the Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Институт неотложной и восстановительной хирургии имени В.К. Гусака Министерства здравоохранения Донецкой Народной Республики</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Institute of Emergency and Reconstructive Surgery named after V.K. Gusak of the Ministry of Health of the Donetsk People’s Republic</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>ГБУ РМЭ «Йошкар-Олинская детская городская больница имени Л.И. Соколовой»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Yoshkar-Ola Children’s City Hospital named after L.I. Sokolova</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>Национальный центр онкологии Министерства здравоохранения Республики Азербайджан</institution><country>Азербайджан</country></aff><aff xml:lang="en"><institution>National Oncology Center of the Ministry of Health of the Republic of Azerbaijan</institution><country>Azerbaijan</country></aff></aff-alternatives><aff-alternatives id="aff-5"><aff xml:lang="ru"><institution>ГАУЗ «Детская республиканская клиническая больница Министерства здравоохранения Республики Татарстан»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Children’s Republican Clinical Hospital of the Ministry of Health of the Republic of Tatarstan</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2023</year></pub-date><pub-date pub-type="epub"><day>28</day><month>12</month><year>2023</year></pub-date><volume>68</volume><issue>4</issue><fpage>432</fpage><lpage>442</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Волкова А.С., Валиев Т.Г., Батманова Н.А., Юлдашева С.А., Гордеева З.С., Сабанцев С.Л., Алексерова Г.А., Осипова И.В., Варфоломеева С.Р., 2023</copyright-statement><copyright-year>2023</copyright-year><copyright-holder xml:lang="ru">Волкова А.С., Валиев Т.Г., Батманова Н.А., Юлдашева С.А., Гордеева З.С., Сабанцев С.Л., Алексерова Г.А., Осипова И.В., Варфоломеева С.Р.</copyright-holder><copyright-holder xml:lang="en">Volkova A.S., Valiev T.T., Batmanova N.A., Yuldasheva S.A., Gordeeva Z.S., Sabantsev S.L., Aleskerova G.A., Osipova I.V., Varfolomeeva S.R.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://www.htjournal.ru/jour/article/view/485">https://www.htjournal.ru/jour/article/view/485</self-uri><abstract><p>Введение. Анапластическая крупноклеточная лимфома (АККЛ) характеризуется клинической, морфологической и иммуногистохимической гетерогенностью. В терапии применяют как интенсивные блоковые схемы, так и программы, аналогичные используемым в лечении острого лимфобластного лейкоза (ОЛЛ). С учетом прогностически неблагоприятного влияния экспрессии Т-клеточных маркеров клетками опухоли был разработан протокол «АККЛ НИИ ДОиГ 2003», учитывающий не только группы риска, но и иммунофенотипические особенности опухолевого субстрата.Цель: оценить эффективность лечения детей, больных АККЛ, по протоколу «АККЛ НИИ ДОиГ 2003» и сравнить ее с результатами лечения по протоколу «NHL-BFM 95».Материалы и методы. В исследование включены 100 больных с впервые установленным диагнозом АККЛ, получивших лечение с 2000 по 2023 гг. в пяти федеральных и региональных детских онкогематологических учреждениях. В зависимости от проводимого протокола лечения больные были разделены на 2 группы: I группа — 48 больных, получавших лечение по протоколу «АККЛ НИИ ДОиГ 2003»; II группа включала 52 больных, получавших лечение по протоколу «NHL-BFM 95». Основу протокола «АККЛ НИИ ДОиГ 2003» составили высокоинтенсивные блоковые режимы, аналогичные таковым, используемым в лечении Т-линейного ОЛЛ группы высокого риска. Проводили сравнительную оценку общей выживаемости (ОВ), бессобытийной выживаемости (БСВ) и безрецидивной выживаемости (БРВ) больных в зависимости от терапевтического протокола с использованием программы SPSS 21.0.Результаты. Десятилетняя ОВ у больных I группы составила 95,3 ± 3,3 %, II группы — 82,0 ± 5,4 % (р = 0,037). 10-летняя БСВ у больных I группы была 95,3 ± 3,3 %, II группы — 68,6 ± 6,5 % (р = 0,001). Десятилетняя БРВ у больных I группы составила 97,3 ± 2,7 %, тогда как II группы — 74,4 ± 6,4 % (р = 0,003). Заключение. Дифференцированный иммуноориентированный подход к терапии АККЛ с помощью программы «АККЛ НИИ ДОиГ 2003» позволил достичь более высоких показателей выживаемости больных по сравнению со стандартным протоколом лечения.</p></abstract><trans-abstract xml:lang="en"><p>Introduction. Anaplastic large cell lymphoma (ALCL) is characterized by clinical, morphological and immunohistochemical heterogeneity. Both intensive block regimens and programs similar to those used in the treatment of acute lymphoblastic leukemia (ALL) are used in therapy. Taking into account the prognostically unfavorable effect of the expression of T-cell markers by tumor cells, a protocol ALCL NII DOIG 2003 was developed, which takes into account not only risk groups, but also immunophenotypic features of the tumor substrate.Aim. To evaluate the treatment effectiveness of children with ALCL according to the protocol ALCL NII DOIG 2003 in comparison with the standard protocol NHL-BFM 95.Materials and methods. The study included 100 patients with newly diagnosed ALCL who received treatment from 2000 to 2023 in 5 federal and regional pediatric oncohematology departments. The patients were divided into 2 groups depending on the protocol of therapy: I group — 48 patients treated with protocol ALCL NII DOIG 2003; II group — 52 patients treated according to the NHL-BFM 95 protocol. The basis of the protocol ALCL NII DOIG 2003 was made up of high-intensity block regimes similar to those used in the treatment of high-risk T-precursor ALL. A comparative assessment of the overall, event-free and relapse-free survival of patients was carried out depending on the therapeutic protocol using the SPSS 21.0 program. Results. The 10-year overall survival rate in patients from I group was 95.3 ± 3.3 %, II group — 82.0 ± 5.4 % (p = 0.037). 10- year event-free survival in I group was 95.3 ± 3.3 %, II — 68.6 ± 6.5 % (p = 0.001). 10-year relapse-free survival in I group of patients was 97.3 ± 2.7 %, while in the II group — 74.4 ± 6.4 % (p = 0.003).Conclusion. The obtained results indicate the high effi ciency of a differentiated, immuno-oriented approach to the treatment of ALCL with the ALCL NII DOIG 2003 protocol, making it possible to achieve signifi cantly higher survival rates of patients compared to the standard treatment protocol.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>анапластическая крупноклеточная лимфома</kwd><kwd>иммунофенотип</kwd><kwd>Т-клеточные маркеры</kwd><kwd>химиотерапия</kwd><kwd>дети</kwd><kwd>подростки</kwd></kwd-group><kwd-group xml:lang="en"><kwd>anaplastic large cell lymphoma</kwd><kwd>immunophenotype</kwd><kwd>T-cell markers</kwd><kwd>chemotherapy</kwd><kwd>children</kwd><kwd>adolescents</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Kaseb H., Mukkamalla S.K.R., Rajasurya V. Anaplastic Large Cell Lymphoma. [Updated 2023 Jan 15]. In: StatPearls [Internet]. 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