scholarly journals A case of intravascular lymphoma diagnosed with a primary vitreoretinal lymphoma-like fundus lesion

Author(s):  
Masaki Asakage ◽  
Kazuhiko Umazume ◽  
Hiroyuki Takoi ◽  
Daigo Akahane ◽  
Yasunori Ishibashi ◽  
...  

Abstract Purpose We report a case of intravascular lymphoma with primary vitreoretinal lymphoma-like fundus findings. Case A 61-year-old man with a one-week history of temporal visual field defect in the left eye was referred by a local ophthalmologist to our department. A yellowish-white raised patchy lesion was found in the nasal fundus of the left eye. Vitreoretinal lymphoma was suspected, and vitrectomy was performed in the left eye for diagnostic purpose. However, vitreous interleukin-10 concentration was low and no significant result was obtained. He had fever of around 38 °C, and respiratory failure that started 2 weeks before ophthalmological examination, worsened. Intravascular lymphoma was diagnosed from the results of histopathological examinations of transbronchial lung biopsy, bone marrow biopsy and random skin biopsy. With the start of systemic chemotherapy, the subretinal lesions shrank gradually and systemic condition was stable. However, 5 months after the start of chemotherapy, spread to the central nervous system was observed, and chimeric antigen receptor T cell (CAR-T) therapy was started in another hospital. After the start of CAR-T therapy, the subretinal lesions shrank further. Conclusions Intravascular lymphoma may be accompanied by primary vitreoretinal lymphoma-like intraocular lesions. If intraocular lesions are accompanied by systemic symptoms such as fever of unknown origin, the possibility of intravascular lymphoma should be suspected and systemic work-up should be performed.

Ophthalmology ◽  
2020 ◽  
Vol 127 (7) ◽  
pp. 956-962 ◽  
Author(s):  
David E. Kuo ◽  
Maggie M. Wei ◽  
Jared E. Knickelbein ◽  
Karen R. Armbrust ◽  
Ian Y.L. Yeung ◽  
...  

Blood ◽  
2021 ◽  
Author(s):  
Carole Soussain ◽  
Denis Malaise ◽  
Nathalie Cassoux

Primary vitreoretinal lymphoma (PVRL) is a rare form of primary central nervous system lymphoma (PCNSL) arising in the intraocular compartment without brain involvement. Despite its apparent indolent clinical course, PVRL can cause permanent vision loss and CNS relapse, the major cause of death in PVRL patients. The pathophysiology of PVRL is unknown. As in PCNSL, the transformation of the tumor cells likely originates outside the CNS, before the cells migrate to the eye and proliferate within an immune-permissive microenvironment. PVRL exhibits a biased immunoglobulin repertoire, suggesting underlying antigen selection. The diagnosis remains challenging, requiring close coordination between ophthalmologists and cytologists. Because of their rarity and fragility in the vitreous, lymphoma cells cannot always be identified. Interleukin levels, molecular biology and imaging are used in combination with clinical ophthalmological examination to support the diagnosis of PVRL. Multi-institutional prospective studies are urgently needed to validate the equivocal conclusions regarding treatments drawn from heterogeneous retrospective or small cohort studies. Intravitreal injections of methotrexate or rituximab or local radiotherapy are effective at clearing tumor cells within the eyes but do not prevent CNS relapse. Systemic treatment based on high-dose methotrexate chemotherapy, with or without local treatment, might reduce this risk. At relapse, intensive consolidation chemotherapy followed by stem cell transplantation can be considered. Single-agent ibrutinib, lenalidomide and temozolomide treatments are effective in patients with relapsed PVRL and should be tested as first-line treatments. Therapeutic response assessment based on a clinical examination is improved by measuring cytokine levels but still needs to be refined.


2021 ◽  
Vol 1 (2) ◽  
pp. 69-75
Author(s):  
SATORU KASE ◽  
KENICHI NAMBA ◽  
DAIJU IWATA ◽  
KAZUOMI MIZUUCHI ◽  
TAKAKO ITO ◽  
...  

Background/Aim: This study aimed to demonstrate the clinical outcomes of primary vitreoretinal lymphoma (PVRL). Patients and Methods: Seventeen patients with PVRL who had been treated at Hokkaido University Hospital were enrolled in this study. They were diagnosed based on their cytology, interleukin-10/-6 ratio, and immunoglobulin heavy chain (IgH) gene rearrangement. Results: Diagnostic tests detected cytological malignancy among 14 cases (82.3%), high interleukin-10/-6 ratios among 16 cases (94.1%), and IgH monoclonality in 13 cases (76.5%). Systemic corticosteroids were given to seven (41.2%) patients before their diagnosis of PVRL. Treatments after diagnosis comprised intravitreal methotrexate injection, local radiation, and intravenous chemotherapy for 11, seven, and five cases, respectively. Central nervous system and systemic involvements were observed in nine and one case, respectively, and these complications occurred at 3 to 43 months (mean=16 months) after initial ocular presentation. Conclusion: Many of our patients did not receive any systemic intervention, and almost half of patients with PVRL developed central nervous system involvement during their follow-up period.


2019 ◽  
Vol 33 (1) ◽  
pp. 66-80 ◽  
Author(s):  
Dimitrios Kalogeropoulos ◽  
Georgios Vartholomatos ◽  
Arijit Mitra ◽  
Ibrahim Elaraoud ◽  
Soon Wai Ch'ng ◽  
...  

2018 ◽  
Vol 49 (10) ◽  
pp. e173-e174
Author(s):  
Luisa Pierro ◽  
Alessandro Arrigo ◽  
Giuseppe Casalino ◽  
Elisabetta Miserocchi ◽  
Emanuela Aragona ◽  
...  

2019 ◽  
Vol Volume 13 ◽  
pp. 353-364 ◽  
Author(s):  
Ramesh Venkatesh ◽  
Bharathi Bavaharan ◽  
Padmamalini Mahendradas ◽  
Naresh Kumar Yadav

2019 ◽  
Vol 3 (6) ◽  
pp. 485-487
Author(s):  
Arthi G. Venkat ◽  
Naveen Karthik ◽  
Ashley Lowe ◽  
Sumit Sharma

Purpose: This paper reports a case of an older white woman presenting with recalcitrant bilateral vitreoretinal inflammation that was ultimately proven to be primary vitreoretinal lymphoma by vitreous biopsy who subsequently developed a branch retinal artery occlusion (BRAO) following an intravitreal injection of methotrexate. Methods: Case summary. Results: The patient was treated with serial intravitreal methotrexate injections and subsequently developed a BRAO immediately following her seventh injection. Conclusions: A full systemic evaluation to rule out other causes of the BRAO was negative and given the timing of her symptoms after the injection it was determined that the BRAO was most likely realted to the intravitreal methotrexate injection.


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