Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children30210.24287/1726-1708-2020-19-1-18-26ORIGINAL ARTICLESОРИГИНАЛЬНЫЕ СТАТЬИUse of romiplostim for newly diagnosed immune thrombocytopenia in childrenПрименение ромиплостима при впервые выявленной иммунной тромбоцитопении у детейhttps://orcid.org/0000-0001-8404-1800SuntsovaE. V.СунцоваЕ. В.
Russian Federation

MD, Department of hematopoiesis depressions, myeloid leukemias, rare and inherited diseases, 

117997, Moscow, Samory Mashela st., 1

научный сотрудник отдела депрессий кроветворения, миелоидных лейкозов, редких и наследственных болезней,

117997, Москва, ГСП-7, ул. Саморы Машела, 1

elena.suncova@fccho-moscow.ruhttps://orcid.org/0000-0002-5067-8288ChikvinaI. I.ЧиквинаИ. И.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-7918-680XSadovskayaM. N.СадовскаяМ. Н.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0003-2787-6005KotskayaN. N.КоцкаяН. Н.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0001-7265-0414HachatryanL. A.ХачатрянЛ. А.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0001-7130-8596BaydildinaD. D.БайдильдинаД. Д.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-0813-5626KalininaI. I.КалининаИ. И.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-1258-8281PetrovaU. N.ПетроваУ. Н.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-2057-2036PshonkinA. V.ПшонкинА. В.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-0224-2746LutfullinI. Ya.ЛутфуллинИ. Я.
Russian Federation

Kazan

Казань

https://orcid.org/0000-0002-6569-6390LemazinaE. N.ЛемазинаЕ. Н.
Russian Federation

Kazan

Казань

https://orcid.org/0000-0001-9833-5156OsipovaI. V.ОсиповаИ. В.
Russian Federation

Kazan

Казань

GaleevaA. V.ГалееваА. В.
Russian Federation

Kazan

Казань

https://orcid.org/0000-0002-6929-7338NaumovaO. S.НаумоваО. С.
Russian Federation

Kazan

Казань

https://orcid.org/0000-0001-9050-7822FisynI. V.ФисюнИ. В.
Russian Federation

Oryol

Орел

https://orcid.org/0000-0002-6552-4257BikovaG. V.БыковаГ. В.
Russian Federation

Stavropol

Ставрополь

https://orcid.org/0000-0002-1881-7444KazaryanG. R.КазарянГ. Р.
Russian Federation

Nizhnevartovsk

Нижневартовск

https://orcid.org/0000-0002-0016-6698MaschanA. A.МасчанА. А.
Russian Federation

Moscow

Москва

https://orcid.org/0000-0002-2322-5734NovichkovaG. A.НовичковаГ. А.
Russian Federation

Moscow

Москва

Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава РоссииRegional Children’s HospitalГАУЗ «Городская детская больница № 1»Scientific and Clinical Multidisciplinary Center for Help for Mothers and Children named after Z.I. KruglayaБУЗ Орловской области «Научно-клинический многопрофильный центр медицинской помощи матерям и детям имени З.И. Круглой»Regional Children’s HospitalГБУЗ Ставропольского края «Краевая детская клиническая больница»Regional Children’s HospitalБУ Ханты-Мансийского АО – Югры «Нижневартовская окружная клиническая детская больница»2803202019118262703202027032020Copyright ©; 2020, «D. Rogachev NMRCPHOI»Copyright ©; 2020, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2020«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/302

Immune thrombocytopenia (ITP) is a disease with a heterogeneous clinical manifestation. In the majority of children newly diagnosed ITP is a self-limited benign disorder, while chronic ITP develops rarely. The clinical onset of ITP can occur in very different ways: from nearly invisible skin hemorrhage to severe life-threatening bleeding. Conventional treatments promote a response in most patients, but in a small number of children thrombocytopenia is unresponsive. In this article, we describe our experience of the clinical use of romiplostim in children with severe unresponsive newly diagnosed ITP. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. The severity of bleeding decreased significantly after the start of romiplostim therapy in all cases. Durable complete (platelets > 100 × 109 /l) response was achieved in five out of six patients 4 to 8 weeks after starting therapy. Three children have remained in lasting remission for 1 to 3 years after the discontinuation of romiplostim. There were no adverse events associated with romiplostim.

Иммунная тромбоцитопения (ИТП) – заболевание с гетерогенной клинической картиной. У большинства детей впервые выявленная ИТП является самоограниченным доброкачественным процессом, и хроническая фаза заболевания развивается редко. Клинические проявления в дебюте заболевания сильно различаются: от практически незаметного кожного геморрагического синдрома до тяжелых жизнеугрожающих кровотечений. Общепринятая терапия позволяет достичь тромбоцитарного ответа у большинства пациентов, но у части детей тромбоцитопения оказывается резистентной к проводимой терапии. В статье описан опыт клинического применения ромиплостима у детей с тяжелой резистентной впервые выявленной ИТП. Данное исследование поддержано Независимым этическим комитетом и утверждено решением Ученого совета НМИЦ ДГОИ. Тяжесть геморрагического синдрома значимо уменьшалась после начала терапии ромиплостимом во всех случаях. Длительный полный (тромбоциты > 100 × 109 /л) тромбоцитарный ответ был достигнут у пяти из шести пациентов через 4–8 недель от начала терапии. После прекращения терапии у троих детей сохраняется ремиссия длительностью от 1 до 3 лет. Нежелательных явлений, связанных с применением ромиплостима, не было отмечено.

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