Deák, Gabor Gy

Vitreoretinal lymphoma (VRL) is a rare ocular pathology that is notorious for mimicking chronic uveitis, which is a seemingly benign condition in comparison. The most common form of VRL is the diffuse large B-cell type, and there has been a high mortality rate. This dismal prognosis can be improved significantly if the disease is diagnosed early, but until now there is no consensus on an appropriate diagnostic algorithm. We conducted a retrospective search of PubMed Central® and analyzed results from thirty-three studies that were published between 2011–2021. The chosen studies incorporated some popular testing tools for VRL, and our analyses focused on comparing the average sensitivity of five diagnostic methods. The methods included cytology including ancillary immunohistochemistry, Myeloid Differentiation Factor 88 (MyD88) mutation analysis, polymerase chain reaction (PCR) for monoclonal rearrangements of immunoglobulin heavy chain (IgH) and T-cell Receptor (TCR) genes, flow cytometry, and IL10 and IL6 analysis. Across the varied diagnostic methods employed in thirty-three studies explored in this analysis, MyD88 mutation assay emerged as a strong contender given its sensitivity and low coefficient of variation. There is an imminent need for the introduction of newer assays that can further improve the sensitivity of identifying MyD88 mutation in cancer cells seen in the vitreous.

Purpose: To describe a novel fluorescein angiographic finding in patients with von Hippel–Lindau disease. Methods: Retrospective case series of patients diagnosed with retinal capillary hemangioblastoma (RCH) in the setting of von Hippel–Lindau. Results: We identified six eyes of three patients with von Hippel–Lindau and leaky retinal vessels. All eyes showed segmental diffuse vascular leakage (SDVL) that was seen in the late phase of the angiogram and that originated from third order and more peripheral retinal veins and adjacent capillaries. These vessels did not drain from the RCHs. Segmental diffuse vascular leakage was mainly seen in the mid and far periphery. In some cases, it was located near the RCHs, while in other cases, it was remote. Segmental diffuse vascular leakage was also seen in one eye without RCHs. On follow-up, the extent and intensity of segmental diffuse vascular leakage did not change after the RCHs were treated with laser or cryotherapy. Conclusion: Diffuse vascular leakage from retinal venules around and away from RCHs in patients with von Hippel–Lindau disease is seen, but the clinical and prognostic importance of this finding is uncertain.