nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.17650/2311-1267-2015-1-54-60nodgo-79Research ArticleОригинальные исследованияТромбоцитопатииThrombocytopathyДёминаИрина АндреевнаDeminaI. A.

Россия, 117198, Москва, ул. Саморы Машела, 1

1 Samory Mashela St., Moscow, 117198, Russia

idemina@mail.ruКумсковаМ. А.KumskovaM. A.

Россия, 117198, Москва, ул. Саморы Машела, 1

1 Samory Mashela St., Moscow, 117198, Russia

ПантелеевМ. А.PanteleevM. A.

Россия, 117198, Москва, ул. Саморы Машела, 1;

 Россия, 125167, Москва, Новый Зыковский проезд, 4а;

Россия, 119991, Москва, Ленинский просп., 38А, корп. 1;

Россия, 119234, Москва, ул. Ленинские Горы, 1, стр. 8

1 Samory Mashela St., Moscow, 117198, Russia;

4a Novo-Zykovskiy Pr-d, Moscow, 125167, Russia; 

38A, Bldg. 1, Leninskiy Prosp., Moscow, 119991, Russia;

1, Bldg. 8 Leninskie Gory St., Moscow, 119234, Russia

ФГБУ «Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава РоссииРоссияFederal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Ministry of Health of RussiaRussian FederationФГБУ «Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России; ФГБУ «Гематологический научный центр» Минздрава России; Центр теоретических проблем физико-химической фармакологии РАН; ФГБОУ ВПО «Московский государственный университет им. М.В. Ломоносова»РоссияFederal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Ministry of Health of Russia; Hematological Research Center, Ministry of Health of Russia; Theoretical Problems Center of Physical and Chemical Pharmacology, Russian Academy of Sciences; M.V. Lomonosov Moscow State UniversityRussian Federation201523052015215460Copyright © Дёмина И.А., Кумскова М.А., Пантелеев М.А., 20152015Дёмина И.А., Кумскова М.А., Пантелеев М.А.Demina I.A., Kumskova M.A., Panteleev M.A.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/79

Статья посвящена общим вопросам классификации и дифференциальной диагностики тромбоцитопатий. Освещены особенности патогенеза, течения и диагностики некоторых редких наследственных тромбоцитопатий. Особое внимание уделено молекулярным основам формирования данных заболеваний. Приведены основные генетические мутации, ассоциированные с рядом тромбоцитопатий.

The article is devoted to general issues of classification and differential diagnosis of thrombocytopathy. Features of pathogenesis, clinical course and diagnosis of certain rare inherited thrombocytopathies are highlighted. Particular attention is paid to the formation of the molecular basis of these diseases. The basic genetic mutations associated with a number of thrombocytopathies are given.

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