Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis—a review of current knowledge and future directions
Primary vitreoretinal lymphoma (PVRL), a rare subtype of primary central nervous system lymphoma (PCNSL), can lead to permanent vision loss and central nervous system (CNS) involvement, resulting in a poor prognosis. PVRL often masquerades as uveitis, and its partial response to topical corticostero...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Health
2025-06-01
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| Series: | Blood Science |
| Online Access: | http://journals.lww.com/10.1097/BS9.0000000000000233 |
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| author | Si-Yu Wang Suo-Wang Zhou Jing Gao Liang Wang |
| author_facet | Si-Yu Wang Suo-Wang Zhou Jing Gao Liang Wang |
| author_sort | Si-Yu Wang |
| collection | DOAJ |
| description | Primary vitreoretinal lymphoma (PVRL), a rare subtype of primary central nervous system lymphoma (PCNSL), can lead to permanent vision loss and central nervous system (CNS) involvement, resulting in a poor prognosis. PVRL often masquerades as uveitis, and its partial response to topical corticosteroids further complicates the diagnosis. The gold standard for diagnosis is cytological analysis; however, owing to its low sensitivity, cytokine profiling and genetic testing may serve as supplementary diagnostic tools. There is no universally accepted consensus regarding PVRL treatment protocols. Combined systemic high-dose intravenous methotrexate (MTX) and intravitreal therapy may help manage bilateral ocular lesions, although this combination’s ability to delay CNS relapse remains controversial. For relapsed or refractory (R/R) PVRL patients aged <60 years, intensive consolidation chemotherapy followed by autologous stem cell transplantation may be considered. Novel targeted therapies such as ibrutinib and lenalidomide have demonstrated efficacy in R/R cases. Large-scale multicenter prospective studies are urgently needed to determine optimal treatment strategies. |
| format | Article |
| id | doaj-art-edf79caa521b4dc0a14f616ea780cd9b |
| institution | OA Journals |
| issn | 2543-6368 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Wolters Kluwer Health |
| record_format | Article |
| series | Blood Science |
| spelling | doaj-art-edf79caa521b4dc0a14f616ea780cd9b2025-08-20T01:57:01ZengWolters Kluwer HealthBlood Science2543-63682025-06-0172e0023310.1097/BS9.0000000000000233202506000-00015Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis—a review of current knowledge and future directionsSi-Yu Wang0Suo-Wang Zhou1Jing Gao2Liang Wang3a Capital Medical University, Beijing, Chinab Aier Eye Hospital, Jinan University, Guangzhou 510071, Chinac Department of Hematology, Beijing Tongren Hospital, Capital Medical University, Beijing, Chinac Department of Hematology, Beijing Tongren Hospital, Capital Medical University, Beijing, ChinaPrimary vitreoretinal lymphoma (PVRL), a rare subtype of primary central nervous system lymphoma (PCNSL), can lead to permanent vision loss and central nervous system (CNS) involvement, resulting in a poor prognosis. PVRL often masquerades as uveitis, and its partial response to topical corticosteroids further complicates the diagnosis. The gold standard for diagnosis is cytological analysis; however, owing to its low sensitivity, cytokine profiling and genetic testing may serve as supplementary diagnostic tools. There is no universally accepted consensus regarding PVRL treatment protocols. Combined systemic high-dose intravenous methotrexate (MTX) and intravitreal therapy may help manage bilateral ocular lesions, although this combination’s ability to delay CNS relapse remains controversial. For relapsed or refractory (R/R) PVRL patients aged <60 years, intensive consolidation chemotherapy followed by autologous stem cell transplantation may be considered. Novel targeted therapies such as ibrutinib and lenalidomide have demonstrated efficacy in R/R cases. Large-scale multicenter prospective studies are urgently needed to determine optimal treatment strategies.http://journals.lww.com/10.1097/BS9.0000000000000233 |
| spellingShingle | Si-Yu Wang Suo-Wang Zhou Jing Gao Liang Wang Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis—a review of current knowledge and future directions Blood Science |
| title | Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis—a review of current knowledge and future directions |
| title_full | Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis—a review of current knowledge and future directions |
| title_fullStr | Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis—a review of current knowledge and future directions |
| title_full_unstemmed | Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis—a review of current knowledge and future directions |
| title_short | Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis—a review of current knowledge and future directions |
| title_sort | primary vitreoretinal lymphoma diagnosis treatment and prognosis a review of current knowledge and future directions |
| url | http://journals.lww.com/10.1097/BS9.0000000000000233 |
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