Мастоцитоз (обзор литературы и описание клинических случаев)

Авторы:
  • А. Л. Меликян
    ФГБУ "Гематологический научный центр" Минздрава России, Москва
  • И. Н. Суборцева
    ФГБУ "Гематологический научный центр" Минздрава России, Москва
  • С. Р. Горячева
    ФГБУ "Гематологический научный центр" Минздрава России, Москва
  • Т. И. Колошейнова
    ФГБУ "Гематологический научный центр" Минздрава России, Москва
  • М. В. Вахрушева
    ФГБУ "Гематологический научный центр" Минздрава России, Москва
  • А. М. Ковригина
    ФГБУ "Гематологический научный центр" Минздрава России, Москва
  • А. Б. Судариков
    ФГБУ "Гематологический научный центр" Минздрава России, Москва
  • В. Н. Двирнык
    ФГБУ "Гематологический научный центр" Минздрава России, Москва
  • Т. Н. Обухова
    ФГБУ "Гематологический научный центр" Минздрава России, Москва
Журнал: Терапевтический архив (архив до 2018 г.). 2014;86(12): 127-134
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Аннотация. Термин "мастоцитоз" объединяет группу редких заболеваний, характеризующихся опухолевой пролиферацией клональных тучных клеток (ТК) и инфильтрацией одного или нескольких органов. Клиническая картина мастоцитоза (МЦ) крайне разнородна - от поражений кожи, которые могут спонтанно регрессировать до агрессивных форм заболевания, ассоциированных с органной недостаточностью и короткой продолжительностью жизни. В настоящее время в классификации ВОЗ (версия 2008 г.) выделено 7 подтипов МЦ. Заболевание диагностируется на основании клинических проявлений и выявления инфильтратов опухолевых ТК с использованием морфологического, иммуногистохимического, иммунофенотипического, генетического и молекулярного методов. Аномальные ТК характеризуются атипичной морфологией и аномальной экспрессией антигенов CD25 и CD2. Повышение активности триптазы в сыворотке является общим признаком для всех подтипов МЦ. Более чем у 90% пациентов имеется мутация D816VKIT в ТК. В данной статье представлен обзор литературы. В качестве клинического примера приведены описания 3 клинических случая у пациентов с различными подтипами МЦ.
Ключевые слова:
  • кожный мастоцитоз
  • системный мастоцитоз
  • тучноклеточный лейкоз
  • мутация D816VKIT
  • иматиниб

КАК ЦИТИРОВАТЬ:

Меликян А.Л., Суборцева И.Н., Горячева С.Р., Колошейнова Т.И., Вахрушева М.В., Ковригина А.М., Судариков А.Б., Двирнык В.Н., Обухова Т.Н. Мастоцитоз (обзор литературы и описание клинических случаев). Терапевтический архив (архив до 2018 г.). 2014;86(12):127-134. https://doi.org/10.17116/terarkh20148612127-134

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