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<article 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" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="review-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Annals of the Russian academy of medical sciences</journal-id><journal-title-group><journal-title xml:lang="en">Annals of the Russian academy of medical sciences</journal-title><trans-title-group xml:lang="ru"><trans-title>Вестник Российской академии медицинских наук</trans-title></trans-title-group></journal-title-group><issn publication-format="print">0869-6047</issn><issn publication-format="electronic">2414-3545</issn><publisher><publisher-name xml:lang="en">"Paediatrician" Publishers LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">1410</article-id><article-id pub-id-type="doi">10.15690/vramn1410</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>INFECTIOUS DISEASES: CURRENT ISSUES</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>АКТУАЛЬНЫЕ ВОПРОСЫ ИНФЕКЦИОННЫХ БОЛЕЗНЕЙ</subject></subj-group><subj-group subj-group-type="article-type"><subject>Review Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">COVID-19-associated secondary hemophagocytic lymphohistiocytosis (cytokine storm syndrome)</article-title><trans-title-group xml:lang="ru"><trans-title>COVID-19-ассоциированный вторичный гемофагоцитарный лимфогистиоцитоз (синдром «цитокинового шторма»)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3874-4721</contrib-id><contrib-id contrib-id-type="spin">4713-9943</contrib-id><name-alternatives><name xml:lang="en"><surname>Alekseeva</surname><given-names>Ekaterina I.</given-names></name><name xml:lang="ru"><surname>Алексеева</surname><given-names>Екатерина Иосифовна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, PhD, Professor, Corresponding Member of the RAS</p></bio><bio xml:lang="ru"><p>д.м.н., профессор, член-корреспондент РАН</p></bio><email>alekatya@yandex.ru</email><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6667-9472</contrib-id><contrib-id contrib-id-type="spin">3907-3234</contrib-id><name-alternatives><name xml:lang="en"><surname>Tepaev</surname><given-names>Rustem F.</given-names></name><name xml:lang="ru"><surname>Тепаев</surname><given-names>Рустэм Фаридович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, PhD</p></bio><bio xml:lang="ru"><p>д.м.н.</p></bio><email>tepaev@nczd.ru</email><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0369-9593</contrib-id><contrib-id contrib-id-type="spin">5826-6385</contrib-id><name-alternatives><name xml:lang="en"><surname>Shilkrot</surname><given-names>Ilia Y.</given-names></name><name xml:lang="ru"><surname>Шилькрот</surname><given-names>Илья Юрьевич</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, PhD</p></bio><bio xml:lang="ru"><p>к.м.н.</p></bio><email>7606399@gmail.com</email><xref ref-type="aff" rid="aff3"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8165-6401</contrib-id><contrib-id contrib-id-type="spin">7524-7085</contrib-id><name-alternatives><name xml:lang="en"><surname>Dvoryakovskaya</surname><given-names>Tatyana M.</given-names></name><name xml:lang="ru"><surname>Дворяковская</surname><given-names>Татьяна Маратовна</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, PhD</p></bio><bio xml:lang="ru"><p>д.м.н.</p></bio><email>bzarova@nczd.ru</email><xref ref-type="aff" rid="aff4"/><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6823-0273</contrib-id><contrib-id contrib-id-type="spin">2805-9855</contrib-id><name-alternatives><name xml:lang="en"><surname>Surkov</surname><given-names>Aleksander G.</given-names></name><name xml:lang="ru"><surname>Сурков</surname><given-names>Александр Геннадьевич</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, PhD</p></bio><bio xml:lang="ru"><p>к.м.н.</p></bio><email>asurkov@1msmu.ru</email><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2370-3182</contrib-id><name-alternatives><name xml:lang="en"><surname>Kriulin</surname><given-names>Ivan A.</given-names></name><name xml:lang="ru"><surname>Криулин</surname><given-names>Иван Алексеевич</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>PhD Student</p></bio><bio xml:lang="ru"><p>Аспирант</p></bio><email>kriulin.vanya@yandex.ru</email><xref ref-type="aff" rid="aff2"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">National Medical Research Center for Children’s Health</institution></aff><aff><institution xml:lang="ru">Национальный медицинский исследовательский центр здоровья детей</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">I.M. Sechenov First Moscow State Medical University (Sechenov University)</institution></aff><aff><institution xml:lang="ru">Первый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)</institution></aff></aff-alternatives><aff-alternatives id="aff3"><aff><institution xml:lang="en">National Association of Non-Governmental Medical Organizations</institution></aff><aff><institution xml:lang="ru">Национальная ассоциация негосударственных медицинских организаций</institution></aff></aff-alternatives><aff-alternatives id="aff4"><aff><institution xml:lang="en">National Medical Research Center for Children’s Health, Moscow</institution></aff><aff><institution xml:lang="ru">Национальный медицинский исследовательский центр здоровья детей</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2021-04-12" publication-format="electronic"><day>12</day><month>04</month><year>2021</year></pub-date><volume>76</volume><issue>1</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>51</fpage><lpage>66</lpage><history><date date-type="received" iso-8601-date="2020-08-04"><day>04</day><month>08</month><year>2020</year></date><date date-type="accepted" iso-8601-date="2021-03-11"><day>11</day><month>03</month><year>2021</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2021, "Paediatrician" Publishers LLC</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2021, Издательство "Педиатръ"</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="en">"Paediatrician" Publishers LLC</copyright-holder><copyright-holder xml:lang="ru">Издательство "Педиатръ"</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/" start_date="2022-04-12"/></permissions><self-uri xlink:href="https://vestnikramn.spr-journal.ru/jour/article/view/1410">https://vestnikramn.spr-journal.ru/jour/article/view/1410</self-uri><abstract xml:lang="en"><p>In most cases, COVID-19 has a favorable outcome. However, the risk of developing critical forms of the disease, including secondary hemophagocytic lymphohistiocytosis – HLH (cytokine storm syndrome), remains high. This dictates the interest in studying pathogenetic mechanisms, features of the clinical picture, laboratory and instrumental criteria for covid-19 disease. The article analyzes the causes of acute respiratory distress syndrome and multiple organ failure as manifestations of HLH. The necessity of monitoring signs of hyperinflammation (ferritin, C-reactive protein, etc., biomarkers of inflammation) and activation of thrombosis is substantiated, in order to make timely decisions about the beginning of pathogenetic therapy. However, there are limitations for routine testing of the level of Pro-inflammatory cytokines. Information about the diagnostic criteria of HLH is summarized, and the expediency of these criteria for establishing secondary HLH, which has complicated the course of COVID-19, is emphasized.</p></abstract><trans-abstract xml:lang="ru"><p>В большинстве случаев COVID-19 отличается благоприятным исходом. Однако риск развития критических форм болезни, в том числе вторичного гемофагоцитарного лимфогистиоцитоза – ГЛГ (синдрома «цитокинового шторма»), остается высоким. Этим продиктован интерес к изучению патогенетических механизмов, особенностей клинической картины, лабораторных и инструментальных критериев тяжелого COVID-19. В статье проанализированы причины развития острого респираторного дистресс-синдрома и полиорганной недостаточности как проявлений ГЛГ. Обоснована необходимость мониторирования признаков гипервоспаления (ферритина, С-реактивного белка и пр. биомаркеров воспаления) и активации тромбообразования, для принятия своевременных решений о начале патогенетической терапии. Вместе с тем, отмечены ограничения для рутинного тестирования уровня провоспалительных цитокинов. Обобщены сведения о диагностических критериях ГЛГ, подчеркнута целесообразность этих критериев для установления вторичного ГЛГ, осложнившего течение COVID-19.</p></trans-abstract><kwd-group xml:lang="en"><kwd>COVID-19</kwd><kwd>«cytokine storm»</kwd><kwd>hemophagocytic lymphohistiocytosis</kwd><kwd>macrophage activation syndrome</kwd><kwd>hyperinflammation</kwd><kwd>pathogenesis</kwd><kwd>early predictors</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>COVID-19</kwd><kwd>«цитокиновый шторм»</kwd><kwd>гемофагоцитарный лимфогистиоцитоз</kwd><kwd>синдром активации макрофагов</kwd><kwd>гипервоспаление</kwd><kwd>патогенез</kwd><kwd>ранние предикторы</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Naming the coronavirus disease (COVID-19) and the virus that causes it. 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