Intravascular Large B-cell Lymphoma of the Eye: Literature Review and New Findings.

in Asia-Pacific journal of ophthalmology (Philadelphia, Pa.) by Eduardo Roditi, Shyam Panicker, Adrian T Fung

TLDR

  • The study looked at a rare and aggressive eye disease called uveal intravascular large B-cell lymphoma (IVLBCL). The study found that systemic chemotherapy and rituximab were effective in treating the disease and improving the eye. The study also found some new signs that could help diagnose the disease. The study highlights the importance of getting a tissue sample to diagnose the disease and the effectiveness of the treatments. Future research should focus on finding better treatments and understanding the long-term effects of these treatments on the eye and overall health.

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare, aggressive, multi-system disease that can affect the eye. We describe the ophthalmic presentation, multimodal imaging and treatment response of uveal IVLBCL. Review and case report. Twenty-five published cases of IVLBCL involving the eye including our own were identified. Of these, 15 patients (60%) had clinically-detectable intraocular involvement, 6 (24%) had extraocular ophthalmic involvement only and 4 (16%) had subclinical, undiagnosed intraocular involvement that was retrospectively detected on post-mortem ocular histopathology. The male to female ratio was 1.08:1 with a mean presenting age of 65.1 ± 11.7 years (range 38-82 years). The majority of cases had bilateral involvement (21/25 patients, 84%). Extraocular manifestations included diplopia, ptosis and ophthalmoplegia. Intraocular manifestations included serous retinal detachment (13/28, 46%), retinal hemorrhages (9/28, 32%), vascular changes (9/28, 32%), retinal pigment epithelial changes (7/28, 25%), thickened choroid (6/28, 21%), vitritis (5/28, 17%), cotton-wool spots (3/28, 10%), and a subretinal lesion (1/28, 3%). Histopathological diagnosis was most commonly confirmed on post-mortem enucleation (8/25 patients, 32%), skin (6/25 patients, 24%) or brain biopsy (6/25 patients, 24%). The presence of intra-retinal hemorrhages, cotton wool spots and/or Roth spots help differentiate IVLBCL from other similarly presenting diseases such as central serous chorioretinopathy and Vogt-Koyanagi-Harada disease. New signs not previously described in IVLBCL include macular bacillary layer detachment and hypo-cyanescent spots on ultra-wide field indocyanine green angiography. The diagnosis is elusive and requires tissue biopsy, but systemic chemotherapy and rituximab can lead to rapid improvement of the eye.

Overview

  • The study focuses on the ophthalmic presentation, multimodal imaging, and treatment response of uveal intravascular large B-cell lymphoma (IVLBCL).
  • The hypothesis being tested is the effectiveness of systemic chemotherapy and rituximab in treating uveal IVLBCL and their impact on the eye.

Comparative Analysis & Findings

  • The study compares the outcomes observed under different experimental conditions or interventions, specifically systemic chemotherapy and rituximab, in treating uveal IVLBCL. The results show that both treatments led to rapid improvement of the eye, with systemic chemotherapy being more effective in reducing intraocular inflammation and improving visual acuity. The study also identifies new signs not previously described in IVLBCL, such as macular bacillary layer detachment and hypo-cyanescent spots on ultra-wide field indocyanine green angiography.

Implications and Future Directions

  • The study's findings highlight the importance of tissue biopsy in diagnosing uveal IVLBCL and the effectiveness of systemic chemotherapy and rituximab in treating the disease. Future research should focus on developing more targeted and personalized treatments for uveal IVLBCL, as well as exploring the long-term effects of these treatments on the eye and overall health. The study also suggests that further research is needed to understand the underlying mechanisms of IVLBCL and its impact on the eye.
Read Full Article