Vitreoretinal lymphoma followed by systemic diffuse large B cell lymphoma

Abstract

Vitreoretinal lymphoma as the presenting diagnosis in association with a systemic lymphoma without central nervous system involvement is exceedingly rare, and the classification of this condition is not well-established. Here, we describe a patient with intermittent blurry vision in the left eye for 2 years in the setting of a recent incidental diagnosis of diffuse large B cell lymphoma from an axillary lymph node biopsy. The diagnosis of panuveitis with an extensive exudative retinal detachment was made. The patient was treated with pars plana vitrectomy as well as systemic chemotherapy, intrathecal methotrexate, intravitreal methotrexate, and intravitreal rituximab with good post-operative outcomes.

Cytologic and Molecular Diagnostics for Vitreoretinal Lymphoma: Current Approaches and Emerging Single-Cell Analyses

“…VRL with an underlying BCL2 translocation was first reported by White et al ( 1999 ) in 1993 and was later reported to be found in 57% of patients with VRL (Wallace et al, 2006 ). Given that most VRL cases are of the ABC DLBCL subtype (Araujo and Coupland, 2017 ; Karakawa et al, 2018 ; Chen et al, 2019 ; Fan et al, 2019 ) and that BCL2 is more commonly found in GCB DLBCL (Barrans et al, 2002 ; Iqbal et al, 2004 ; Chen et al, 2010 ), the high occurrence of the BCL2 translocation in patients with VRL as reported by Wallace et al ( 2006 ) is intriguing (Fend et al, 2016 ; Kalogeropoulos et al, 2019 ). In a cohort of 106 patients with DLBCL, both overall survival and progression-free survival were significantly poorer in 27 patients who had the BCL2 translocation (Zhang et al, 2011 ).…”

“…Based on this stratification according to molecular signatures, PCNSL was subsequently classified as ABC DLBCL (Camilleri-Broet et al, 2006 ; Bhagavathi et al, 2008 ; Cheng et al, 2008 ; Montesinos-Rongen et al, 2009 ; Hattab et al, 2010 ; Li et al, 2017 ; Belhouachi et al, 2020 ). Indeed, >80% of VRL cases belong to this subtype (Araujo and Coupland, 2017 ; Karakawa et al, 2018 ; Chen et al, 2019 ; Fan et al, 2019 ).…”

Systemic lymphoma masquerading as Vogt-Koyanagi-Harada syndrome: Report of a case with multimodal imaging and histopathology

“…1 Rarely, it can present with vitreoretinal involvement. 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 We report a unique case of systemic lymphoma presenting with neurologic symptoms and bilateral exudative retinal detachments suggestive of Vogt-Koyanagi-Harada (VKH) syndrome, expanding the spectrum of ocular manifestations of systemic lymphoma.…”

Table_1.DOCX

“…VRL with an underlying BCL2 translocation was first reported by White et al (1999) in 1993 and was later reported to be found in 57% of patients with VRL (Wallace et al, 2006). Given that most VRL cases are of the ABC DLBCL subtype (Araujo and Coupland, 2017;Karakawa et al, 2018;Chen et al, 2019;Fan et al, 2019) and that BCL2 is more commonly found in GCB DLBCL (Barrans et al, 2002;Iqbal et al, 2004;Chen et al, 2010), the high occurrence of the BCL2 translocation in patients with VRL as reported by Wallace et al (2006) is intriguing (Fend et al, 2016;Kalogeropoulos et al, 2019). In a cohort of 106 patients with DLBCL, both overall survival and progression-free survival were significantly poorer in 27 patients who had the BCL2 translocation (Zhang et al, 2011).…”

“…Based on this stratification according to molecular signatures, PCNSL was subsequently classified as ABC DLBCL (Camilleri-Broet et al, 2006;Cheng et al, 2008;Montesinos-Rongen et al, 2009;Hattab et al, 2010;Li et al, 2017;Belhouachi et al, 2020). Indeed, >80% of VRL cases belong to this subtype (Araujo and Coupland, 2017;Karakawa et al, 2018;Chen et al, 2019;Fan et al, 2019).…”