bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2026-71-2-211-225bloodjour-764Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCASE REPORTSПо следам дебютных катастроф: первичная витреоретинальная лимфомаFollowing debut disasters: primary vitreoretinal lymphomahttps://orcid.org/0000-0001-5762-8294КоролеваД. А.KorolevaD. A.

Королева Дарья Александровна, кандидат медицинских наук, гематолог отделения гематологии и химиотерапии лимфом с блоком трансплантации костного мозга и гемопоэтических стволовых клеток; ассистент кафедры гематологии лечебного факультета НОИ клинической медицины им. Н.А. Семашко

125167, г. Москва

127473, г. Москва

Daria A. Koroleva, Cand. Sci. (Med.), Hematologist, Department of Hematology and Chemotherapy of Lymphomas with Bone Marrow and Hematopoietic Stem Cell Transplantation Unit; Assistant Professor, Department of Hematology, Faculty of Medicine, Scientific Research Institute of Clinical Medicine N.A. Semashko

125167, Moscow

127473, Moscow

koroleva_12-12@mail.ruhttps://orcid.org/0000-0002-2639-7419ЗвонковЕ. Е.ZvonkovE. E.

Звонков Евгений Евгеньевич, доктор медицинских наук, заведующий отделением гематологии и химиотерапии лимфом с блоком трансплантации костного мозга и гемопоэтических стволовых клеток; профессор кафедры гематологии лечебного факультета НОИ клинической медицины им. Н.А. Семашко

125167, г. Москва

127473, г. Москва

Evgeny E. Zvonkov, Dr. Sci. (Med), Head of the Department of Hematology and Chemotherapy of Lymphomas with Bone Marrow and Hematopoietic Stem Cell Transplantation Unit; Professor of the Department of Hematology, Faculty of Medicine, Scientific Research Institute of Clinical Medicine N.A. Semashko

125167, Moscow

127473, Moscow

dr.zvonkov@gmail.comЩецоваО. О.ShchetsovaO. O.

Щецова Ольга Олеговна, гематолог отделения гематологии и химиотерапии лимфом с блоком трансплантации костного мозга и гемопоэтических стволовых клеток

125167, г. Москва

Olga O. Shchetsova, Hematologist, Department of Hematology and Chemotherapy of Lymphomas with Bone Marrow and Hematopoietic Stem Cell Transplantation Unit

125167, Moscow

ole4ka_89-11@mail.ruhttps://orcid.org/0000-0002-3585-7595СмирноваА. Б.SmirnovaA. B.

Смирнова Анна Борисовна, офтальмолог клинико-диагностического отделения гематологии и миелоидных неоплазий

125167, г. Москва

Anna B. Smirnova, Ophthalmologist, Department of Hematology and MyeloidNeoplasia

125167, Moscow

anja03@yandex.ruhttps://orcid.org/0000-0003-3914-8611НикулинаЕ. Е.NikulinaE. E.

Никулина Елена Евгеньевна, научный сотрудник лаборатории молекулярной гематологии

125167, г. Москва

Elena E. Nikulina, Research Associate Laboratory of Molecular Hematology

125167, Moscow

nikulina.e@blood.ruhttps://orcid.org/0000-0002-7036-9968СеверинаН. А.SeverinaN. A.

Северина Наталья Александровна, кандидат биологических наук, врач молекулярно-генетической диагностики

125167, г. Москва

Nataliya A. Severina, Cand. Sci. (Biol.), Physician, Clinical Laboratory of Molecular Genetic Diagnostics

125167, Moscow

severina.n@blood.ruhttps://orcid.org/0000-0001-9463-9187СудариковА. Б.SudarikovA. B.

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

125167, г. Москва

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

125167, Moscow

dusha@blood.ruhttps://orcid.org/0000-0002-7335-5633БорисоваД. В.BorisovaD. V.

Борисова Дарья Владимировна, заведующая подразделением терапевтического лечения макулярной патологии

428028, г. Чебоксары

Darya V. Borisova, Head of the Department of Therapeutic Treatment of Macular Pathology

428028, Cheboksary

darya.sychyova.94@mail.ruhttps://orcid.org/0000-0003-4213-4923ВоскресенскаяА. А.VoskresenskayaA. A.

Воскресенская Анна Александровна, кандидат медицинских наук, заместитель директора по научной работе

428028, г. Чебоксары

Anna A. Voskresenskaya, Cand. Sci. (Med.), Deputy Director for Research

428028, Cheboksary

vsolaris@mail.ruhttps://orcid.org/0000-0003-3637-3645ПоздееваН. А.PozdeevaN. A.

Поздеева Надежда Александровна, доктор медицинских наук, директор

428028, г. Чебоксары

Nadezhda A. Pozdeyeva, Dr. Sci. (Med.), Head

428028, Cheboksary

npozdeeva@mail.ruhttps://orcid.org/0000-0003-2668-9136ГришинаЕ. Е.GrishinaE. E.

Гришина Елена Евгеньевна, доктор медицинских наук, профессор, офтальмоонколог, главный научный сотрудник онкологического отделения хирургических методов лечения, профессор кафедры офтальмологии и оптометрии факультета усовершенствования врачей

129110, г. Москва

Elena E. Grishina, Dr. Sci. (Med.), Professor, Ophthalmooncologist, Chief Research Officer of the Oncological Department of Surgical Methods, Professor of the Department of Ophthalmology and Optometry, Faculty of Advanced Studies for Doctors of the Moscow Regional Research and Clinical Institute named after M.F. Vladimirsky

129110, Moscow

eyelena@mail.ruhttps://orcid.org/0000-0002-7242-8781АндрюхинаО. М.AndryukhinaO. M.

Андрюхина Ольга Михайловна, кандидат медицинских наук, врач-офтальмолог, старший научный сотрудник офтальмологического отделения, доцент кафедры офтальмологии и оптометрии факультета усовершенствования врачей

129110, г. Москва

Olga M. Andryukhina, Cand. Sci. (Med.), Ophthalmologist, Senior Research Assistant in the ophthalmology department, Associate Professor of the Department of Ophthalmology and Optometry at the Faculty of Advanced Studies for Doctors of the Moscow Regional Research and Clinical Institute named after M.F.Vladimirsky

129110, Moscow

a-olya86@mail.ruhttps://orcid.org/0009-0009-7005-2002МаксимоваЕ. М.MaksimovaE. M.

Максимова Екатерина Максимовна, младший научный сотрудник офтальмологического отделения, врач-офтальмолог

129110, г. Москва

Ekaterina M. Maksimova, Junior Researcher in the Ophthalmology Department,Ophthalmologist of the Moscow Regional Research and Clinical Institutenamed after M.F. Vladimirsky

129110, Moscow

katyamef@mail.ruhttps://orcid.org/0000-0001-6177-3566ПаровичниковаЕ. Н.ParovichnikovaE. N.

Паровичникова Елена Николаевна, член-корреспондент РАН, доктор медицинских наук, генеральный директор; заведующая кафедрой гематологии лечебного факультета НОИ клинической медицины им. Н.А. Семашко

125167, г. Москва

127473, г. Москва

Elena N. Parovichnikova, Corresponding Member of RAS, Dr. Sci. (Med.); Head of the Department of Hematology, Faculty of Medicine, Scientific Research Institute of Clinical Medicine named after N.A. Semashko

125167, Moscow

127473, Moscow

ФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской Федерации; ФГБОУ ВО «Российский университет медицины» Министерства здравоохранения Российской ФедерацииРоссияNational Research Center for Hematology; Russian University of MedicineRussian FederationФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Research Center for HematologyRussian FederationЧебоксарский филиал ФГАУ НМИЦ «МНТК “Микрохирургия глаза” им. академика С.Н. Федорова» Министерства здравоохранения Российской ФедерацииРоссияThe Cheboksary Branch of the S. Fyodorov Eye Microsurgery Federal State InstitutionRussian FederationГБУЗ МО «Московский областной научно-исследовательский клинический институт им. М.Ф. Владимирского»РоссияMoscow Regional Research and Clinical Institute (“MONIKI”)Russian Federation202617072026712211225Copyright © Королева Д.А., Звонков Е.Е., Щецова О.О., Смирнова А.Б., Никулина Е.Е., Северина Н.А., Судариков А.Б., Борисова Д.В., Воскресенская А.А., Поздеева Н.А., Гришина Е.Е., Андрюхина О.М., Максимова Е.М., Паровичникова Е.Н., 20262026Королева Д.А., Звонков Е.Е., Щецова О.О., Смирнова А.Б., Никулина Е.Е., Северина Н.А., Судариков А.Б., Борисова Д.В., Воскресенская А.А., Поздеева Н.А., Гришина Е.Е., Андрюхина О.М., Максимова Е.М., Паровичникова Е.Н.Koroleva D.A., Zvonkov E.E., Shchetsova O.O., Smirnova A.B., Nikulina E.E., Severina N.A., Sudarikov A.B., Borisova D.V., Voskresenskaya A.A., Pozdeeva N.A., Grishina E.E., Andryukhina O.M., Maksimova E.M., Parovichnikova E.N.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/764Введение

Введение. Первичная витреоретинальная лимфома (ПВРЛ) относится к подтипу лимфом иммунопривилегированных органов. Проблемой является как диагностика, так и выбор тактики терапии ПВРЛ.

Цель

Цель: представить клиническое наблюдение больных ПВРЛ и возможности улучшения диагностики и результатов лечения.

Основные сведения

Основные сведения. С 2024 г. в ФГБУ «НМИЦ гематологии» проведено лечение двух больных ПВРЛ в возрасте 66 и 75 лет. Диагноз был установлен на основании молекулярного анализа мутаций в генах MYD88/CD79b в водянистой влаге стекловидного тела. Индукция ремиссии проведена по протоколу «R-MPV» с добавлением леналидомида и ибрутиниба, затем выполнена консолидация в виде ауто-ТГСК, режим кондиционирования по программе «ВВС». На ранних сроках после ауто-ТГСК у обеих больных констатирован рецидив заболевания. Таким образом, для улучшения диагностики целесообразно внедрение малоинвазивных методов исследований молекулярного профиля ПВРЛ. Рациональна интеграция инновационной клеточной терапии в качестве первой линии лечения и использование режимов кондиционирования на основе тиотепы.

Introduction

Introduction. Primary vitreoretinal lymphoma (PVRL) is a subtype of lymphoma of immune-privileged organs. Both diagnosis and treatment decisions pose significant challenges for PVRL.

Aim

Aim: to present a clinical observation in treating patients with PVRL and possibilities for improving diagnosis and treatment outcomes.

Main Findings

Main Findings. Since 2024, two patients with PVRL, aged 66 and 75 years, have been treated at the National Medical Research Center for Hematology. Modern diagnostic methods based on molecular analysis to detect mutations in the MYD88/ CD79b genes in vitreous fluid were used to establish the diagnosis. A two-stage approach was used for treatment: remission induction using the R-MPV protocol with the addition of lenalidomide and ibrutinib, followed by consolidation auto-HCT in a conditioning regimen according to the “BBC” program. Disease relapses were detected in both patients early after auto-HCT. Thus, for timely diagnosis, it is advisable to implement minimally invasive methods for studying the molecular profile of PVRL. The development of new therapeutic approaches, integrating innovative cell therapy into first-line treatment and the use of thiotepa-based conditioning regimens, is also advisable.

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