Успешная терапия периферической Т-клеточной лимфомы, ассоциированной с энтеропатией, с применением высокодозной полихимиотерапии и трансплантации аутологичных стволовых кроветворных клеток - Журнал Терапевтический архив №7 Вопросы гематологии 2013

Успешная терапия периферической Т-клеточной лимфомы, ассоциированной с энтеропатией, с применением высокодозной полихимиотерапии и трансплантации аутологичных стволовых кроветворных клеток

Воробьев В.И., Кравченко С.К., Ковригина А.М. Успешная терапия периферической Т-клеточной лимфомы, ассоциированной с энтеропатией, с применением высокодозной полихимиотерапии и трансплантации аутологичных стволовых кроветворных клеток. Терапевтический архив (архив до 2018 г.). 2013;85(7):79-83.

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Аннотация

Т-клеточная лимфома, ассоциированная с энтеропатией (ТЛАЭ), — редкое заболевание, которое составляет не более 1,4% от всех лимфом. Наиболее часто встречается в Европе, реже в Северной Америке и Азии. В 50% случаев ассоциирована с чувствительной к глютену целиакией, подразделяется на I и II типы. Применение среднедозной CHOP-подобной терапии неэффективно — медиана общей продолжительности жизни составляет 7—10 мес. Высокодозная терапия позволяет достичь 60% 5-летней выживаемости, но ее использование возможно не более чем в половине случаев. Это связано с тяжелым соматическим статусом большинства больных на момент диагностики и медианой возраста 57—64 года. В статье представлен обзор литературы и успешный случай лечения 58-летнего пациента с ТЛАЭ I типа с использованием протокола mNHL-BFM-90 и аутологичной трансплантации стволовых кроветворных клеток.

Ключевые слова: Т-клеточная лимфома, ассоциированная с энтеропатией, целиакия, NHL-BFM-90, трансплантация ауто-логичных стволовых кроветворных клеток.

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Enteropathy-associated T-cell lymphoma (EATL) is a rare disease that accounts for not more than 1.4% of all lymphomas. It is most common in Europe, followed by North America and Asia. The disease is associated with gluten-sensitive celiac disease in 50% of cases and divided into types I and II. Mean-dose CHOP-like therapy is ineffective, with a median overall survival of 7—10 months. With high-dose therapy, 5-year survival rates can be 60%, but it can be used in not more than half of the cases. This is associated with the serious somatic status of most patients at diagnosis and with a median age of 57—64 years. The article presents a literature review and a case of successful therapy in a 58-year-old patient with type I EATL using the mNHL-BFM-90 protocol and autologous hematopoietic stem cell transplantation.

Key words: enteropathy-associated T-cell lymphoma, celiac disease, NHL-BFM-90, autologous hematopoietic stem cell trans-plantation.

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Авторы

В.И. ВОРОБЬЕВ, С.К. КРАВЧЕНКО, А.М. КОВРИГИНА

Гематологический научный центр Минздрава России, Москва

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V.I. VOROBYEV, S.K. KRAVCHENKO, A.M. KOVRIGINA

Hematology Research Center, Ministry of Health of Russia, Moscow

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