Современные подходы к диагностике и лечению эссенциальной тромбоцитемии: обзор литературы и собственные данные

РЕДКИЕ ГЕМАТОЛОГИЧЕСКИЕ ЗАБОЛЕВАНИЯ И СИНДРОМЫ , , ,

Абдулкадыров К.М., Шуваев В.А., Мартынкевич И.С.

ФГБУ «Российский научно-исследовательский институт гематологии и трансфузиологии Федерального медико-биологического агентства», ул. 2-я Советская, д. 16, Санкт-Петербург, Российская Федерация, 191024

Для переписки: Василий Анатольевич Шуваев, канд. мед. наук, ул. 2-я Советская, д. 16, Санкт-Петербург, Российская Федерация, 191024; тел.: +7(921)636-54-72; e-mail: shuvaev77@mail.ru

Для цитирования: Абдулкадыров К.М., Шуваев В.А., Мартынкевич И.С. Современные подходы к диагностике и лечению эссенциальной тромбоцитемии: обзор литературы и собственные данные. Клиническая онкогематология. 2015;8(3):235–47.

РЕФЕРАТ

Цель. Обзор литературы с освещением вопросов эпидемиологии, терминологии, этиологии, механизмов становления и развития эссенциальной тромбоцитемии, а также представление собственных данных.

Методы. Описываются молекулярно-генетические основы патогенеза заболевания и клиническая картина его проявлений. Рассматриваются вопросы, касающиеся диагностических критериев, дифференциальной диагностики и классификации эссенциальной тромбоцитемии. Приведены результаты молекулярно-генетических и цитогенетических исследований, основные причины развития тромботических осложнений, методы их профилактики и лечения. Широко освещаются методы лечения, мониторинг и оценка эффективности терапии заболевания. Представлен анализ собственного опыта диагностики и лечения 218 взрослых пациентов с эссенциальной тромбоцитемией. Женщин было 161, мужчин — 57, соотношение по полу 2,8:1, медиана возраста составила 57,2 года (диапазон 18,3–89,3 года). Хромосомные аберрации выявлены у 7 (9,3 %) из 65 обследованных цитогенетически пациентов. Мутация JAK2V617F обнаружена у 79 (58,1 %) из 136 больных, а гена MPL — у 1 (2,3 %) из 44.

Результаты. Общая 10-летняя выживаемость 218 больных эссенциальной тромбоцитемией составила 83,9 %. Прогрессирование в фазу вторичного посттромбоцитемического миелофиброза зарегистрировано у 13 (6 %) пациентов. Лечение осуществлялось гидроксимочевиной (n = 132), интерферонами-a (n = 37), анагрелидом (n = 10), ацетилсалициловой кислотой (n = 54). Летальность за весь период наблюдения в течение 10 лет составила 16,1 % (n = 35).

Заключение. Своевременная и ранняя диагностика эссенциальной тромбоцитемии, определение тактики терапии с учетом риска тромбоэмболических осложнений и последующее динамическое наблюдение за больными с контролем уровня тромбоцитов служат залогом сохранения продолжительности и качества жизни пациентов.

Ключевые слова: эссенциальная тромбоцитемия, мутация в гене Янус-киназы 2 (JAK2), риск тромботических осложнений, алгоритм диагностики и лечения.

Получено: 14 января 2015 г.

Принято в печать: 26 мая 2015 г.

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