RESUMEN
Purpose: To report a case of a perifoveal exudative vascular anomalous complex (PEVAC) resembling lesion in a patient with multiple myeloma. Observations: A 56-year-old male with multiple myeloma presented with sudden moderate vision loss in the right eye. Best-corrected visual acuity was 20/25 in his right eye. Fundus examination showed a vascular irregularity in the perifoveal region. Fluorescein angiography (FA) revealed an isolated perifoveal aneurysmal lesion with minimal leakage. On optical coherence tomography (OCT) examination, a large oval structure with a hyperreflective wall and exudation was visualised. Three weeks later, spontaneous improvement of the intraretinal fluid was observed on OCT without treatment. However, 3 months later the macular edema recurred. The appearance of the aneurysmal lesion is similar to a PEVAC lesion, which is an isolated well-defined perifoveal intraretinal vascular abnormality presenting on OCT as a round hyperreflective structure with a dark lumen containing variably reflective material and is commonly associated with cystic intraretinal fluid. Conclusions and Importance: PEVAC was originally described as occurring in healthy patients, but recent observations suggest that it also appears in association with other retinal/choroidal vascular abnormalities or underlying cardiovascular abnormalities. Our case supports this hypothesis by demonstrating a PEVAC resembling lesion in a patient with multiple myeloma.
RESUMEN
Purpose: Diabetic retinopathy (DR) is a major microvascular complication of type 2 diabetes mellitus (T2DM). Myelomonocytic proangiogenic cells (PAC) have been implicated in DR pathogenesis, but their functional and developmental abnormalities are unclear. In this study we assessed PAC characteristics from healthy controls, T2DM patients with DR (DR) and without (NoDR) in order to determine the consequence of the diabetic condition on PAC phenotype and function, and whether these differ between DR and NoDR patients. Methods: PAC were generated by culturing PBMC on fibronectin coating and then immunophenotyped using flow cytometry. Furthermore, cells were sorted based on CD14, CD105, and CD133 expression and added to an in vitro 3-D endothelial tubule formation assay, containing GFP-expressing human retinal endothelial cells (REC), pericytes, and pro-angiogenic growth factors. Tubule formation was quantified by fluorescence microscopy and image analysis. Moreover, sorted populations were analyzed for angiogenic mediator production using a multiplex assay. Results: The expression of CD16, CD105 and CD31, but not CD133, was lower in PAC from T2DM patients with or without DR. Myeloid and non-myeloid T2DM-derived sorted populations increased REC angiogenesis in vitro as compared to control cultures. They also showed increased S100A8 secretion, decreased VEGF-A secretion, and similar levels of IL-8, HGF, and IL-3 as compared to healthy control (HC)-derived cell populations. Conclusion: T2DM PAC are phenotypically and functionally altered compared to PAC from HC. Differences between DR and NoDR PAC are limited. We propose that impaired T2DM PAC provide inadequate vascular support and promote compensatory, albeit pathological, retinal neovascularization.