nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2020-7-3-86-93nodgo-630Research ArticleПРАКТИЧЕСКИЕ ВОПРОСЫ ДЕТСКОЙ ОНКОЛОГИИ-ГЕМАТОЛОГИИPRACTICAL QUESTIONS OF PEDIATRIC ONCOLOGY-HEMATOLOGYТромботическая микроангиопатия, ассоциированная с трансплантацией гемопоэтических стволовых клеток: общая характеристика и пример из клинической практикиThrombotic microangiopathy associated with hematopoietic stem cell transplantation: general characteristics and an example from clinical practicehttps://orcid.org/0000-0003-2395-4045МачневаЕ. Б.MachnevaE. B.

Елена Борисовна Мачнева, кандидат медицинских наук, врач-гематолог отделения трансплантации костного мозга и гемопоэтических стволовых клеток НИИ детской онкологии и гематологии НМИЦ онкологии им. Н.Н. Блохина, врач-гематолог отделения детской трансплантации костного мозга Российской детской клинической больницы РНИМУ им. Н.И. Пирогова

 115478, Москва, Каширское шоссе, 23,

117997, Москва, Ленинский просп., 117

Cand. of Sci. (Med.), Hematologist Department of Pediatric Bone Marrow and Hematopoietic Stem Cell Transplantation of N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia, Hematologist Department of Bone Marrow Transplantation at the Russian Children’s Clinical Hospital of the N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia

23 Kashirskoe Shosse, Moscow, 115478,

117 Leninskiy Prosp., Moscow, 117997

lena.machneva@yandex.ruhttps://orcid.org/0000-0003-4933-8750БолохоноваМ. А.BolokhonovaM. A.

Клинический ординатор НИИ детской онкологии и гематологии

 115478, Москва, Каширское шоссе, 23

Clinical Resident of the Research Institute of Pediatric Oncology and Hematology

23 Kashirskoe Shosse, Moscow, 115478

masha151922@mail.ruhttps://orcid.org/0000-0003-1091-1521АлиевТ. З.AlievT. Z.

Врач-детский онколог отделения детской трансплантации костного мозга и гемопоэтических стволовых клеток НИИ детской онкологии и гематологии

 115478, Москва, Каширское шоссе, 23

Pediatric Oncologist Department of Pediatric Bone Marrow and Hematopoietic Stem Cell Transplantation

23 Kashirskoe Shosse, Moscow, 115478

timaliev118@gmail.comhttps://orcid.org/0000-0002-7439-4431ШевцовД. В.ShevtsovD. V.

Врач-детский онколог отделения детской трансплантации костного мозга и гемопоэтических стволовых клеток НИИ детской онкологии и гематологии

 115478, Москва, Каширское шоссе, 23

Pediatric Oncologist Department of Pediatric Bone Marrow and Hematopoietic Stem Cell Transplantation

23 Kashirskoe Shosse, Moscow, 115478

denis.shevtsov86@gmail.comhttps://orcid.org/0000-0002-5489-1879СулеймановаА. М.SuleymanovaA. M.

Врач-детский онколог отделения детской трансплантации костного мозга и гемопоэтических стволовых клеток НИИ детской онкологии и гематологии

 115478, Москва, Каширское шоссе, 23

Pediatric Oncologist Department of Pediatric Bone Marrow and Hematopoietic Stem Cell Transplantation

23 Kashirskoe Shosse, Moscow, 115478

a.sulejmanova@ronc.ruhttps://orcid.org/0000-0003-3797-5808СидороваН. В.SidorovaN. V.

Заведующая отделением детской трансплантации костного мозга и гемопоэтических стволовых клеток НИИ детской онкологии и гематологии

 115478, Москва, Каширское шоссе, 23

Head of the Department of Pediatric Bone Marrow and Hematopoietic Stem Cell Transplantation

23 Kashirskoe Shosse, Moscow, 115478

valerevna25@mail.ruhttps://orcid.org/0000-0002-3067-1601ОсмановЕ. А.OsmanovE. A.

Доктор медицинских наук, профессор, заведующий отделом гематологии и трансплантации костного мозга

 115478, Москва, Каширское шоссе, 23

Dr. of Sci. (Med.), Professor, Head of the Department of Hematology and Bone Marrow Transplantation

23 Kashirskoe Shosse, Moscow, 115478

89031538878@mail.ruhttps://orcid.org/0000-0002-2945-284XКиргизовК. И.KirgizovK. I.

Кандидат медицинских наук, заместитель директора по научной и образовательной работе НИИ детской онкологии и гематологии

 115478, Москва, Каширское шоссе, 23

Cand. of Sci. (Med.), Deputy Director for Scientific and Educational Work of Research Institute of Pediatric Oncology and Hematology

23 Kashirskoe Shosse, Moscow, 115478

k.kirgizov@ronc.ruФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России; РДКБ ФГАОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава РоссииРоссияN.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia; Russian Children’s Clinical Hospital of the N.I. Pirogov Russian National Research Medical University, Ministry of Health of RussiaRussian FederationФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава РоссииРоссияN.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of RussiaRussian Federation202020092020738693Copyright © Мачнева Е.Б., Болохонова М.А., Алиев Т.З., Шевцов Д.В., Сулейманова А.М., Сидорова Н.В., Османов Е.А., Киргизов К.И., 20202020Мачнева Е.Б., Болохонова М.А., Алиев Т.З., Шевцов Д.В., Сулейманова А.М., Сидорова Н.В., Османов Е.А., Киргизов К.И.Machneva E.B., Bolokhonova M.A., Aliev T.Z., Shevtsov D.V., Suleymanova A.M., Sidorova N.V., Osmanov E.A., Kirgizov K.I.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nodgo.org/jour/article/view/630

Ассоциированная с трансплантацией гемопоэтических стволовых клеток (ТГСК), или трансплантационно-ассоциированная тромботическая микроангиопатия (ТА-ТМА), в настоящее время является общепризнанным и тяжелым осложнением ТГСК с высоким риском летальности. ТМА характеризуется микроангиопатической гемолитической анемией и тромбоцитопенией, возникающими в результате скопления тромбоцитов в микроциркуляторном русле, что приводит к дисфункции ишемизированного органа. В основе патогенеза ТА-ТМА лежит повреждение эндотелия различными триггерными факторами (в частности, химиотерапевтические агенты в режиме кондиционирования, применение ингибиторов кальциневрина, аллореактивность, инфекционные агенты), вследствие чего создаются провоспалительный и протромботический статусы, а также запускаются классический и альтернативный пути активации системы комплемента. В статье представлены основные особенности терминологии, патогенеза, клинических проявлений ТА-ТМА, а также существующие в настоящее время терапевтические стратегии, применяемые при данной патологии. На клиническом примере продемонстрированы основные проблемы ведения пациентов с ТА-ТМА.

Associated hematopoietic stem cell transplantation (HSCT) or transplant-associated thrombotic microangiopathy (TA-TMA) is currently a generally recognized and severe complication of HSCT with a high risk of mortality. TMA is characterized by microangiopathic hemolytic anemia and thrombocytopenia, resulting in the accumulation of platelets in the microvasculature, which leads to dysfunction of the ischemic organ. The pathogenesis of TА-TMA is based on endothelial damage by various trigger factors (in particular, chemotherapeutic agents in the conditioning regimen, the use of calcineurin inhibitors, alloreactivity, infectious agents). The article presents the peculiarities of terminology, pathogenesis and clinical manifestations of TA-TMA, methods of therapy for this pathology. Examples of management of patients with TA-TMA are demonstrated using a clinical example.

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