Лечение агрессивного системного мастоцитоза и тучноклеточного лейкоза. Обзор литературы и серия клинических наблюдений
ISSN (print) 1997-6933     ISSN (online) 2500-2139
PDF_2024-17-2_180-194

Ключевые слова

мастоцитозы
триптаза
тучные клетки
тучноклеточный лейкоз
ген KIT
кладрибин
иматиниб

Как цитировать

1.
Потапенко В.Г., Морозова Е.В. Лечение агрессивного системного мастоцитоза и тучноклеточного лейкоза. Обзор литературы и серия клинических наблюдений. Клиническая онкогематология. 2024;17(2):180-194. doi:10.21320/2500-2139-2024-17-2-180-194

Ключевые слова

Аннотация

Цель. Представить серию клинических наблюдений течения и терапии агрессивного системного мастоцитоза (АСМ) и тучноклеточного лейкоза (ТКЛ).

Материалы и методы. В работе представлены истории болезни взрослых пациентов с АСМ (n = 4) и ТКЛ (n = 2), получавших циторедуктивное лечение преимущественно в Городской клинической больнице № 31 (Санкт-Петербург). Больные были в возрасте 36–61 год (медиана 50 лет); женщин было 4, мужчин — 2.

Результаты. У всех больных достигнут стойкий ответ на лечение кладрибином (n = 3), иматинибом (n = 2), комбинацией леналидомида, элотузумаба и дексаметазона с последующей трансплантацией аллогенных гемопоэтических стволовых клеток (n = 1). При медиане наблюдения 124 мес. (диапазон 55–186 мес.) 1 из 2 пациентов с ТКЛ умер на фоне прогрессирования сопутствующего гиперэозинофильного синдрома и вторичного инфекционного осложнения.

Заключение. Терапия кладрибином у 2 пациентов с АСМ и иматинибом у 2 пациентов с ТКЛ привела к стойкому многолетнему ответу с сохранением удовлетворительного качества жизни. Необходимы сравнительные исследования для оценки эффективности и определения оптимальной последовательности применения различных противоопухолевых препаратов.

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