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. KorolevaRussian 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
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
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
Russian Federation
Anna B. Smirnova, Ophthalmologist, Department of Hematology and Myeloid
Neoplasia
125167, Moscow
E. E. Nikulina
Russian Federation
Elena E. Nikulina, Research Associate Laboratory of Molecular Hematology
125167, Moscow
N. A. Severina
Russian Federation
Nataliya A. Severina, Cand. Sci. (Biol.), Physician, Clinical Laboratory of Molecular Genetic Diagnostics
125167, Moscow
A. B. Sudarikov
Russian Federation
Andrey B. Sudarikov, Dr. Sci. (Biol.), Head of Department of Molecular Hematology
125167, Moscow
D. V. Borisova
Russian Federation
Darya V. Borisova, Head of the Department of Therapeutic Treatment of Macular Pathology
428028, Cheboksary
A. A. Voskresenskaya
Russian Federation
Anna A. Voskresenskaya, Cand. Sci. (Med.), Deputy Director for Research
428028, Cheboksary
N. A. Pozdeeva
Russian Federation
Nadezhda A. Pozdeyeva, Dr. Sci. (Med.), Head
428028, Cheboksary
E. E. Grishina
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
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
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
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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Review
For citations:
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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