Мантийноклеточная лимфома: история, современные принципы диагностики, лечение (обзор литературы)

ОБЗОРЫ

Г.С. Тумян

ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России, Каширское ш., д. 24, Москва, Российская Федерация, 115478

Для переписки: Гаяне Сепуговна Тумян, д-р мед. наук, Каширское ш., д. 24, Москва, Российская Федерация, 115478; e-mail: gaytum@mail.ru

Для цитирования: Тумян Г.С. Мантийноклеточная лимфома: история, современные принципы диагностики, лечение (обзор литературы). Клиническая онкогематология. 2020;13(4):366–81.

DOI: 10.21320/2500-2139-2020-13-4-366-381

РЕФЕРАТ

Мантийноклеточная лимфома (МКЛ) — гетерогенное заболевание с широким спектром клинических проявлений от редких индолентных случаев, не требующих немедленного начала терапии, до агрессивных, быстро пролиферирующих типов опухоли. Разное клиническое поведение имеет молекулярное обоснование, которое позволило в последней редакции классификации опухолей кроветворной и лимфоидной тканей ВОЗ разделить МКЛ на два варианта: классический (в большинстве случаев) и индолентный. Последние десятилетия расширили наши представления о биологии и механизмах развития заболевания. Это делает возможным в дальнейшем стратифицировать больных на разные группы риска не только в зависимости от клинических факторов (MIPI), но и с учетом молекулярных и биологических особенностей опухоли (индекс пролиферации Ki-67, мутации генов ТР53, NOTCH1, NOTCH2, комплексный кариотип, немутантный статус IGHV). Алгоритмы лечения, основанные на интенсивной химиотерапии с включением цитарабина в высоких дозах, трансплантации аутологичных гемопоэтических стволовых клеток с дальнейшей поддерживающей терапией ритуксимабом, позволили длительно контролировать заболевание у значительного числа больных МКЛ. Применение новых «chemo-free» режимов и рациональных комбинаций (бортезомиб, ингибиторы BTK, леналидомид, венетоклакс) вселяет надежду на возможность ухода от традиционной химиотерапии у определенной части пациентов. Появление новых лекарственных средств с уникальными механизмами действия позволили в какой-то степени снять «печать фатальности» с МКЛ.

Ключевые слова: мантийноклеточная лимфома, лечение.

Получено: 17 июня 2020 г.

Принято в печать: 2 сентября 2020 г.

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