Successful therapy for enteropathy-associated peripheral T-cell lymphoma using high-dose polychemotherapy and autologous hematopoietic stem cell transplantation


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Abstract

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.

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