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Following debut disasters: primary vitreoretinal lymphoma

https://doi.org/10.35754/0234-5730-2026-71-2-211-225

Abstract

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

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.

About the Authors

D. A. Koroleva
National Research Center for Hematology; Russian University of Medicine
Russian Federation

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



E. E. Zvonkov
National Research Center for Hematology; Russian University of Medicine
Russian Federation

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



O. O. Shchetsova
National Research Center for Hematology
Russian Federation

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

125167, Moscow



A. B. Smirnova
National Research Center for Hematology
Russian Federation

Anna B. Smirnova, Ophthalmologist, Department of Hematology and Myeloid
Neoplasia

125167, Moscow



E. E. Nikulina
National Research Center for Hematology
Russian Federation

Elena E. Nikulina, Research Associate Laboratory of Molecular Hematology

125167, Moscow



N. A. Severina
National Research Center for Hematology
Russian Federation

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

125167, Moscow



A. B. Sudarikov
National Research Center for Hematology
Russian Federation

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

125167, Moscow



D. V. Borisova
The Cheboksary Branch of the S. Fyodorov Eye Microsurgery Federal State Institution
Russian Federation

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

428028, Cheboksary



A. A. Voskresenskaya
The Cheboksary Branch of the S. Fyodorov Eye Microsurgery Federal State Institution
Russian Federation

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

428028, Cheboksary



N. A. Pozdeeva
The Cheboksary Branch of the S. Fyodorov Eye Microsurgery Federal State Institution
Russian Federation

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

428028, Cheboksary



E. E. Grishina
Moscow Regional Research and Clinical Institute (“MONIKI”)
Russian Federation

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



O. M. Andryukhina
Moscow Regional Research and Clinical Institute (“MONIKI”)
Russian Federation

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



E. M. Maksimova
Moscow Regional Research and Clinical Institute (“MONIKI”)
Russian Federation

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

129110, Moscow



E. N. Parovichnikova
National Research Center for Hematology; Russian University of Medicine
Russian Federation

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



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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. Following debut disasters: primary vitreoretinal lymphoma. Russian journal of hematology and transfusiology. 2026;71(2):211-225. (In Russ.) https://doi.org/10.35754/0234-5730-2026-71-2-211-225

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ISSN 0234-5730 (Print)
ISSN 2411-3042 (Online)