Department of Ophthalmology, Byers Eye Institute, Stanford University School of Medicine, Palo Alto, California.
Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan Health System, Ann Arbor, Michigan.
Ophthalmol Retina. 2020 Mar;4(3):300-309. doi: 10.1016/j.oret.2019.09.018. Epub 2019 Oct 9.
To describe clinical findings, laboratory values, and treatment response of patients with monoclonal gammopathy of undetermined significance (MGUS) demonstrating neurosensory macular detachment.
Retrospective case series.
Seven eyes of 4 patients (3 men and 1 woman; age range, 60-81 years) with neurosensory macular detachment, treatment-resistant submacular fluid, and vitelliform material.
We retrospectively reviewed the medical and ocular histories, ocular examination findings, retinal imaging, ocular disease course, and laboratory findings in 4 patients with submacular fluid associated with MGUS.
Description of the macular findings and treatment courses of 4 patients diagnosed with MGUS maculopathy.
Seven eyes of 4 patients demonstrated neurosensory macular detachment with treatment-resistant submacular fluid and vitelliform material. No eyes demonstrated signs of significant hyperviscosity retinopathy. Fluorescein angiography showed no definite leakage in any involved eye. Laboratory evaluation revealed immunoglobulin G MGUS in all 4 patients. All 4 patients were resistant to treatments aimed at resolving the subretinal fluid, including some combination of anti-vascular endothelial growth factor injections, photodynamic therapy, topical dorzolamide, oral dosing of eplerenone or acetazolamide, or some combination thereof. In 3 patients, MGUS underwent malignant transformation 24 to 144 months after diagnosis, in 1 patient to lymphoplasmacytic lymphoma and in 2 patients to multiple myeloma. The fourth patient showed no evidence of malignancy 8 years after diagnosis.
Submacular fluid without fluorescein leakage and unresponsive to conventional treatment may suggest an underlying immunoproliferative disorder that we have termed monoclonal gammopathy of macular significance. Given the propensity for monoclonal gammopathy of macular significance to transform into malignant disease in our series, serum protein analysis should be considered in patients with neurosensory macular detachment not attributable to known causes.
描述表现为神经感觉性黄斑脱离的意义未明单克隆丙种球蛋白血症（MGUS）患者的临床发现、实验室值和治疗反应。
回顾性病例系列。
4 名患者（3 名男性和 1 名女性；年龄范围为 60-81 岁）的 7 只眼，这些患者存在神经感觉性黄斑脱离、对治疗有抗性的黄斑下液和类脂性物质。
我们回顾性地审查了 4 名黄斑下液与 MGUS 相关的患者的医疗和眼部病史、眼部检查结果、视网膜成像、眼部疾病过程和实验室结果。
描述 4 名诊断为 MGUS 黄斑病患者的黄斑发现和治疗过程。
4 名患者的 7 只眼均表现出神经感觉性黄斑脱离，黄斑下液和类脂性物质对治疗有抗性。没有眼显示出明显高粘滞性视网膜病变的迹象。荧光素血管造影术显示，任何受累眼均无明确渗漏。实验室评估显示，4 名患者均存在免疫球蛋白 G MGUS。所有 4 名患者对旨在解决眼内液的治疗均有抗性，包括抗血管内皮生长因子注射、光动力疗法、局部多佐胺、口服依普利酮或乙酰唑胺或其组合的某种组合。在 3 名患者中，MGUS 在诊断后 24 至 144 个月发生恶性转化，1 名患者为淋巴浆细胞淋巴瘤，2 名患者为多发性骨髓瘤。第 4 名患者在诊断后 8 年未显示出恶性肿瘤的证据。
没有荧光素渗漏且对常规治疗无反应的黄斑下液可能提示存在潜在的免疫增殖性疾病，我们将其称为有意义的单克隆丙种球蛋白血症。鉴于我们系列中的有意义的单克隆丙种球蛋白血症倾向于转化为恶性疾病，因此对于无法归因于已知原因的神经感觉性黄斑脱离患者，应考虑进行血清蛋白分析。
