bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2022-68-4-520-529bloodjour-492Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCASE REPORTSПрименение ингибитора МЕК-киназы для лечения волосатоклеточного лейкозаMEK-kinase Inhibitor as a treatment option for hairy cell leukemiaАль-РадиЛ. С.Al-RadiL. S.

Аль-Ради Любовь Саттаровна, кандидат медицинских наук, старший научный сотрудник клинико-диагностического отделения гематологии и химиотерапии

Liubov S. Al-Radi, Cand. Sci. (Med.), Hematologist, Consultative Hematology Department

125167

Moscow

lalradi@gmail.comhttps://orcid.org/0000-0001-6220-8868СмирноваС. Ю.SmirnovaS. Yu.

Смирнова Светлана Юрьевна, кандидат медицинских наук, гематолог клинико-диагностического отделения гематологии и химиотерапии, научный сотрудник лаборатории молекулярной гематологии

Svetlana Yu. Smirnova, Cand. Sci. (Med.), Researcher, Laboratory of Molecular Hematology, Hematologist, Consultative Hematology Department

125167

Moscow

smirnova-s-ju@yandex.ruhttps://orcid.org/0000-0001-9591-8508МоисееваТ. Н.MoiseevaT. N.

Моисеева Татьяна Николаевна, кандидат медицинских наук, заведующая клинико-диагностическим отделением гематологии и химиотерапии

Tatiana N. Moiseeva, Cand. Sci. (Med.), Head of the Consultative Hematology Department

125167

Moscow

taniamoiseeva@mail.ruhttps://orcid.org/0000-0002-5532-1122ЯкутикИ. А.YakutikI. A.

Якутик Игорь Александрович, кандидат медицинских наук, научный сотрудник лаборатории молекулярной гематологии

Igor A. Yakutik, Cand. Sci. (Med.), Researcher

125167

Moscow

igorya90@list.ruhttps://orcid.org/0000-0001-9463-9187СудариковА. Б.SudarikovA. B.

Судариков Андрей Борисович, доктор биологических наук, заведующий лабораторией молекулярной гематологии

Andrey B. Sudarikov, Dr. Sci. (Biol.), Head of Department of Molecular Hematology

125167

Moscow

dusha@blood.ruhttps://orcid.org/0000-0001-9984-389XГурьяноваМ. А.GuryanovaM. A.

Гурьянова Маргарита Анатольевна, гематолог отделения химиотерапии гематологических заболеваний

Margarita A. Gurianova, Hematologist of the Department of Chemotherapy of Неmatological Disorders

125167

Moscow

margarita.samtcova@yandex.ruhttps://orcid.org/0000-0002-4155-7820ГрибановаЕ. О.GribanovaE. O.

Грибанова Елена Олеговна, кандидат медицинских наук, заведующаяотделением химиотерапии гематологических заболеваний

Elena O. Gribanova, Cand. Sci. (Med.), Head of the Department of Intensive High-Dose Chemotherapy for Hematological Diseases

125167

Moscow

gribanova.e@blood.ruhttps://orcid.org/0000-0002-9877-0796ДвирныкВ. Н.DvirnykE. N.

Двирнык Валентина Николаевна, кандидат медицинских наук, заведующая централизованной клинико-диагностической лабораторией

Valentina N. Dvirnik, Cand. Sci. (Med.), Head of the Regional Clinical and Diagnostic Laboratory

125167

Moscow

dvirnyk.v@blood.ruhttps://orcid.org/0000-0002-1082-8659КовригинаА. М.KovriginaA. M.

Ковригина Алла Михайловна, доктор биологических наук, заведующаяпатологоанатомическим отделением ФГБУ «Национальный медицинскийисследовательский центр гематологии»

Alla M. Kovrigina, Dr. Sci. (Biol.), Head of the Pathological Department

125167

Moscow

kovrigina.a@blood.ruФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for HematologyRussian Federation202328122023684520529Copyright © Аль-Ради Л.С., Смирнова С.Ю., Моисеева Т.Н., Якутик И.А., Судариков А.Б., Гурьянова М.А., Грибанова Е.О., Двирнык В.Н., Ковригина А.М., 20232023Аль-Ради Л.С., Смирнова С.Ю., Моисеева Т.Н., Якутик И.А., Судариков А.Б., Гурьянова М.А., Грибанова Е.О., Двирнык В.Н., Ковригина А.М.Al-Radi L.S., Smirnova S.Y., Moiseeva T.N., Yakutik I.A., Sudarikov A.B., Guryanova M.A., Gribanova E.O., Dvirnyk E.N., Kovrigina A.M.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.htjournal.ru/jour/article/view/492

Введение. Волосатоклеточный лейкоз (ВКЛ) — хроническое индолентное В-клеточное лимфопролиферативное заболевание. Несмотря на достигнутые успехи в лечении, остается группа больных с резистентным/рецидивирующим (Р/Р) течением заболевания, короткой ремиссией после проведения лечения, с противопоказаниями к проведению стандартного лечения. Применение ингибитора пути RAS-RAF-MEK-ERK при маркерной мутации V600E гена BRAF может оказаться эффективной опцией лечения при Р/Р течении ВКЛ.Цель — представить результаты применения ингибитора MEK-киназы траметиниба для лечения больных ВКЛ.Основные сведения. Ингибитор MEK-киназы траметиниб применяли в дозе 1 мг/сут в течение 3 мес. у 3 больных. У 2 из 3 больных траметиниб позволил осуществить подготовительный этап перед проведением основного курса лечения кладрибином, у одной больной с Р/Р течением ВКЛ монотерапия траметинибом позволила достичь хорошей частичной ремиссии, продолжается прием траметиниба без ухудшения качества жизни.Заключение. Лечение траметинибом может использоваться как подготовительный этап у больных ВКЛ без мутации BRAF и как основная противоопухолевая терапия у больных с Р/Р ВКЛ. Препарат эффективен даже при отсутствии мутаций гена MAP2K1. Монотерапия траметинибом может быть эффективна при применении препарата в сниженной дозировке (1 мг/сут или 1 мг через день).

Introduction. Hairy cell leukemia (HCL) is a chronic indolent B–cell lymphoproliferative disease with a good response to treatment in most cases. However, despite successful treatment, there remains a group of patients with a resistant/recurrent course of the disease, a short remission after treatment, and with contraindications to standard therapy. The use of the RASRAF- MEK-ERK pathway inhibitor in the V600E marker mutation of the BRAF gene may prove to be an effective treatment option for complicated/resistant cases of HCL.Aim — to present the results of the use MEK-kinase inhibitor trametinib in the treatment of HCL.Main findings. The MEK-kinase inhibitor trametinib was used at a dose of 1 mg/day for 3 months in three patients. In two patients trametinib was used as preliminary stage before the main course of treatment with cladribine. In one patient with a resistant/relapsing course of HCL, trametinib monotherapy made it possible to achieve a good partial remission, and the treatment continues without a decrease in the quality of life.Conclusion. Trametinib can be used as a preliminary stage before analogous purine treatment in patients with HCL without the BRAF V600E mutation, in a case of deep neutropenia or infectious complications, and as the main antitumor therapy in patients with resistant/recurrent HCL. Trametinib is effective in the absence of the MAP2K1 mutations. Trametinib monotherapy can be effective at a reduced dosage (1 mg/day or 1 mg every other day).

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The authors declare that there are no conflicts of interest present.