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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">nodgo</journal-id><journal-title-group><journal-title xml:lang="ru">Российский журнал детской гематологии и онкологии (РЖДГиО)</journal-title><trans-title-group xml:lang="en"><trans-title>Russian Journal of Pediatric Hematology and Oncology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2311-1267</issn><issn pub-type="epub">2413-5496</issn><publisher><publisher-name>LTD “Graphica”</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.17650/2311-1267-2018-5-3-74-88</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-410</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>ОБЗОРЫ ЛИТЕРАТУРЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>LITERATURE REVIEWS</subject></subj-group></article-categories><title-group><article-title>Аспекты методологии лабораторных исследований гемостаза в детской гематологии-онкологии и общие подходы в патологии гемостаза при лейкозах</article-title><trans-title-group xml:lang="en"><trans-title>Аspects of the methodology of laboratory studies of hemostasis in pediatric hematology-oncology and general approaches in the pathology of hemostasis in leukemia</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кольцова</surname><given-names>Е. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Koltsova</surname><given-names>E. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Екатерина Михайловна Кольцова.</p><p>117997, Москва, ул. Саморы Машела, 1; 119991, Москва, ул. Косыгина, 4</p></bio><bio xml:lang="en"><p>1 Samory Mashela St., Moscow, 117997; 4 Kosygina St., Moscow, 119991</p></bio><email xlink:type="simple">ekaterina_koltsova@bk.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>Баландина</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Balandina</surname><given-names>A. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>117997, Москва, ул. Саморы Машела, 1; 119991, Москва, ул. Косыгина, 4</p></bio><bio xml:lang="en"><p>1 Samory Mashela St., Moscow, 117997; 4 Kosygina St., Moscow, 119991</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Серегина</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Seregina</surname><given-names>E. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>1 Samory Mashela St., Moscow, 117997</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Полетаев</surname><given-names>М. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Poletaev</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>1 Samory Mashela St., Moscow, 117997</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Вуймо</surname><given-names>Т. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Vuymo</surname><given-names>T. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>1 Samory Mashela St., Moscow, 117997</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Пантелеев</surname><given-names>М. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Panteleev</surname><given-names>M. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>117997, Москва, ул. Саморы Машела, 1; 119991, Москва, ул. Косыгина, 4; 119992, Москва, ул. Ленинские Горы, 1, стр. 2; 141701, Московская облаcть, Долгопрудный, Институтский пер., 9</p></bio><bio xml:lang="en"><p>1 Samory Mashela St., Moscow, 117997; 4 Kosygina St., Moscow, 119991; 1, Bldg. 2 Leninskie Gory St., Moscow, 119992; 9 Institutskiy Per., Dolgoprudny, Moscow Region, 141701</p></bio><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Атауллаханов</surname><given-names>Ф. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Ataullakhanov</surname><given-names>F. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>117997, Москва, ул. Саморы Машела, 1; 119991, Москва, ул. Косыгина, 4; 119992, Москва, ул. Ленинские Горы, 1, стр. 2; 141701, Московская облаcть, Долгопрудный, Институтский пер., 9</p></bio><bio xml:lang="en"><p>1 Samory Mashela St., Moscow, 117997; 4 Kosygina St., Moscow, 119991; 1, Bldg. 2 Leninskie Gory St., Moscow, 119992; 9 Institutskiy Per., Dolgoprudny, Moscow Region, 141701</p></bio><xref ref-type="aff" rid="aff-3"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России; ФГБУН ЦТП ФХФ Российской академии наук</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia; Center for Theoretical Problems of Physicochemical Pharmacology</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России; ФГБУН ЦТП ФХФ Российской академии наук; ФГБОУ ВО МГУ  им. М.В. Ломоносова; ФГАОУ ВО Московский физико-технический институт (государственный университет)</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia; Center for Theoretical Problems of Physicochemical Pharmacology; Lomonosov Moscow State University; Moscow Institute of Physics and Technology (State University)</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2018</year></pub-date><pub-date pub-type="epub"><day>16</day><month>09</month><year>2018</year></pub-date><volume>5</volume><issue>3</issue><fpage>74</fpage><lpage>88</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Кольцова Е.М., Баландина А.Н., Серегина Е.А., Полетаев М.В., Вуймо Т.А., Пантелеев М.А., Атауллаханов Ф.И., 2018</copyright-statement><copyright-year>2018</copyright-year><copyright-holder xml:lang="ru">Кольцова Е.М., Баландина А.Н., Серегина Е.А., Полетаев М.В., Вуймо Т.А., Пантелеев М.А., Атауллаханов Ф.И.</copyright-holder><copyright-holder xml:lang="en">Koltsova E.M., Balandina A.N., Seregina E.A., Poletaev A.V., Vuymo T.A., Panteleev M.A., Ataullakhanov F.I.</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://journal.nodgo.org/jour/article/view/410">https://journal.nodgo.org/jour/article/view/410</self-uri><abstract><p>Пациенты детского возраста с острыми лейкозами сталкиваются с высокими рисками развития тромботических и геморрагических осложнений. Патогенез нарушений гемостаза при гемобластозах носит сложный характер, поскольку помимо самого заболевания также вносят существенный вклад агрессивность применяемой терапии и необходимость многочисленных инвазивных манипуляций. Больные гемобластозами в равной степени подвержены и тромбозам, и кровотечениям, что позволяет говорить о разнонаправленных сдвигах баланса системы гемостаза у каждого индивидуального пациента. Стандартные лабораторные тесты гемостаза (время свертывания, маркерные тесты) предназначены для оценки концентраций отдельных белков и функционирования отдельных компонентов системы гемостаза и никак не оценивают баланс между ее прокоагулянтными и антикоагулянтными составляющими. Альтернативой стандартной коагулограмме могут послужить глобальные тесты гемостаза, предназначенные для оценки баланса свертывания, такие как тромбоэластография, тест генерации тромбина и тромбодинамика. В обзоре разобраны механизмы работы различных лабораторных тестов гемостаза, а также проведена оценка их информативности при частых осложнениях основного заболевания (сепсис, ведущий к развитию синдрома диссеминированного внутрисосудистого свертывания (ДВС); тромбоцитопения) и катетеризации, которой подвергается большинство пациентов с гемобластозами. Общие скрининговые тесты системы свертывания крови имеют малую диагностическую ценность при ДВС-синдроме, возникающем вследствие сепсиса у больных острыми лейкозами, в основном из-за своей нечувствительности к гиперкоагуляции. Стандартные маркеры (например, D-димеры) неспецифичны и лишь подтверждают клинические проявления нарушения свертывания при сепсисе и септическом шоке, но не в состоянии предсказать динамику развития этого процесса на более ранних стадиях воспалительного ответа. При этом тест генерации тромбина и тромбодинамика позволяют выявить гиперкоагуляционную фазу ДВС-синдрома. Тромбоцитопения сопровождает практически все протоколы химиотерапии. При этом степень кровоточивости не всегда зависит только от концентрации тромбоцитов, поскольку химиотерапевтические препараты могут влиять не только на количество, но и на функциональные характеристики тромбоцитов, которые не определяются при стандартном обследовании пациентов. Катетеризация, сопровождающая лечение гемобластозов, является ведущей причиной тромбозов у детей с острыми лейкозами. Тромбоэмболия легочной артерии вследствие тромбоза в системе центральных вен возникает у 8–15 % пациентов. Предикция катетер-ассоциированных тромбозов с помощью стандартных лабораторных методов оценки состояния системы гемостаза не представляется возможной. Отсутствие в современных схемах обследования чувствительных тестов приводит к тому, что лечащий врач вынужден ориентироваться исключительно на клиническую картину уже случившегося тромбоза или кровотечения. Появление новых функциональных методов оценки гемостаза позволяет думать, что уже сегодня существующая стандартная панель тестов коагулограммы может быть дополнена и сделана гораздо более информативной с точки зрения предикции тромбогеморрагических осложнений в области детской гематологии-онкологии.</p></abstract><trans-abstract xml:lang="en"><p>Children with acute leukemia are faced with high risks of thrombotic and hemorrhagic complications. The pathogenesis of haemostasis disorders in hemoblastoses is complex because, in addition to the disease itself, the aggressiveness of the therapy and the need for numerous invasive manipulations also make a significant contribution. Patients with hemoblastoses are equally susceptible to thrombosis and hemorrhage, which makes it possible to speak of multidirectional shifts in the balance of the hemostatic system in each individual patient. Standard laboratory hemostasis tests (clotting times, marker tests) are designed to assess the concentrations of individual proteins and the functioning of individual components of the hemostasis, and in do not assess the balance between its procoagulant and anticoagulant components. Global hemostatic tests designed to assess the coagulation balance, such as thromboelastography, thrombin generation test, and thrombodynamics, can be the alternative for the standard coagulation assays. The review focuses on the mechanisms of various laboratory hemostasis tests, as well as an assessment of their informative value in frequent complications of the underlying disease (sepsis leading to the development of disseminated intravascular coagulation (DIC) syndrome, thrombocytopenia) and catheterization, which is present in the majority of patients with hemoblastosis. General screening tests of the blood coagulation system have little diagnostic value in the DIC syndrome in patients with acute leukemia, mainly due to their insensitivity to hypercoagulability. Standard markers (for example, D-dimers) are non-specific and only confirm the clinical manifestations of clotting disorder in sepsis and septic shock, but are unable to predict the dynamics of this process at earlier stages of the inflammatory response. In this case, the thrombin generation test and thrombodynamics make it possible to reveal the hypercoagulable phase of the DIC syndrome. Thrombocytopenia accompanies almost all protocols of chemotherapy. In this case, the degree of bleeding does not always depend only on the concentration of platelets, since chemotherapeutic drugs can affect not only the quantity, but also the functional characteristics of platelets, which are not determined by standard examination of patients. The catheterization that accompanies the treatment of hemoblastoses is the leading cause of thrombosis in children with acute leukemia. Thromboembolism of the pulmonary artery due to thrombosis in the central vein system occurs in 8–15 % of patients. The prediction of catheter-associated thromboses using standard laboratory methods for assessing the state of the hemostasis is not possible. Absence of sensitive tests in modern diagnostic schemes leads to the fact that the attending physician is forced to focus exclusively on the clinical picture of thrombosis or bleeding. The development of new functional methods of hemostasis allows one to think that today the existing standard panel of coagulation tests can be expanded and made much more informative in terms of the prediction of thrombohemorrhagic complications in pediatric hematology-oncology.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>гемостаз</kwd><kwd>детская гематология-онкология</kwd><kwd>тромбоз</kwd><kwd>кровотечение</kwd><kwd>лабораторная диагностика</kwd></kwd-group><kwd-group xml:lang="en"><kwd>hemostasis</kwd><kwd>pediatric hematology-oncology</kwd><kwd>thrombosis</kwd><kwd>hemorrhage</kwd><kwd>laboratory diagnostics</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Российский научный фонд, гранты № 16-14-00224, № 17-74-10224; грант президента для молодых ученых МК-913.2017.4; Российский фонд фундаментальных исследований, грант № 17-04-01309</funding-statement><funding-statement xml:lang="en">Russian Science Foundation, grant No. 16-14-00224, No. 17-74-10224; grant from the President for young scientists MK-913.2017.4; Russian Foundation for Basic Research grant No. 17-04-01309</funding-statement></funding-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Karapinar B., Cura A. 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