ген CALR

Биология миелопролиферативных новообразований

МИЕЛОИДНЫЕ ОПУХОЛИ , , , , , , ,

А.Л. Меликян, И.Н. Суборцева

ФГБУ «Гематологический научный центр» Минздрава России, Новый Зыковский пр-д, д. 4а, Москва, Российская Федерация, 125167

Для переписки: Ирина Николаевна Суборцева, канд. мед. наук, Новый Зыковский пр-д, д. 4а, Москва, Российская Федерация, 125167; тел.: +7(495)612-44-71; e-mail: soubortseva@yandex.ru

Для цитирования: Меликян А.Л., Суборцева И.Н. Биология миелопролиферативных новообразований. Клиническая онкогематология. 2016;9(3):314-25.

DOI: 10.21320/2500-2139-2016-9-3-314-325

РЕФЕРАТ

Хронические миелопролиферативные заболевания (ВОЗ, 2001), или миелопролиферативные новообразования/опухоли (МПН) (ВОЗ, 2008), являются клональными заболеваниями, характеризуются пролиферацией одной или более клеточной линии миелопоэза в костном мозге с признаками сохраняющейся терминальной дифференцировки и, как правило, сопровождаются изменениями показателей крови. В группу классических Ph-негативных МПН отнесены истинная полицитемия, эссенциальная тромбоцитемия, первичный миелофиброз и МПН неклассифицируемое. Приобретенные соматические мутации, лежащие в основе патогенеза Ph-негативных МПН, представлены мутациями генов JAK2 (V617F, экзон 12), MPL, CALR. Мутации перечисленных генов наблюдаются примерно у 90 % больных. Однако данные молекулярные события не являются уникальными в патогенезе заболеваний. Мутации других генов (ТЕТ2, ASXL1, CBL, IDH1/IDH2, IKZF1, DNMT3A, SOCS, EZH2, TP53, RUNX1 и HMGA2) принимают участие в формировании фенотипа заболевания. В настоящем обзоре описываются современные представления о молекулярной биологии МПН.

Ключевые слова: хронические миелопролиферативные заболевания, миелопролиферативные новообразования, истинная полицитемия, эссенциальная тромбоцитемия, первичный миелофиброз, ген JAK2, ген CALR, ген MPL.

Получено: 11 апреля 2016 г.

Принято в печать: 11 апреля 2016 г.

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