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载脂蛋白AI源性玻璃体淀粉样变性:难以捉摸的诊断。

Apolipoprotein AI-Derived Vitreous Amyloidosis: An Elusive Diagnosis.

作者信息

Yepez Juan B, Murati Felipe A, Petitto Michele, De Yepez Jazmin, Galue Jose M, Vinardell Susana, Mura Marco, Arevalo J Fernando

机构信息

Vitreoretinal Surgery Department, Clinica de Ojos, Maracaibo, Venezuela.

Glaucoma Department, Clinica de Ojos, Maracaibo, Venezuela.

出版信息

Case Rep Ophthalmol. 2020 Jul 6;11(2):287-292. doi: 10.1159/000508065. eCollection 2020 May-Aug.

Abstract

A 56-year-old female presented with vitreous opacity with gradual visual disturbance in her right eye of 1-year duration. A Non-Hodgkin's lymphoma had been treated 15 years before. Presenting best-corrected visual acuity (BCVA) was 20/200 in her right eye and 20/25 in her left eye. Intraocular pressure was 18 mm Hg bilaterally. Slit-lamp examination revealed no abnormal findings in the anterior segment of both eyes, including the absence of cells and flare. Fundoscopic examination indicated hazy media with the typical glass-wool-like appearance in her right eye. B-scan ultrasound demonstrated that the vitreous was full of middle-echo spots, vitreous opacities, and posterior vitreous detachment occurred. The patient underwent vitreous biopsy and a standard 25-gauge pars plana vitrectomy (diagnostic and therapeutic). Intraoperatively, the eye was noted to have severe diffuse debris and very strong vitreoretinal adhesions. Cytospin smears prepared from the vitreous aspirate indicated amorphous acellular material that stained positively with Congo Red and showed apple green birefringence on polarized microscopy, consistent with the diagnosis of amyloidosis. A genetic evaluation of tongue tissue demonstrated apolipoprotein AI-derived amyloidosis. The BCVA was 20/25 OU at 3 months postoperatively.

摘要

一名56岁女性因右眼玻璃体混浊伴逐渐加重的视力障碍就诊,病程1年。15年前曾接受非霍奇金淋巴瘤治疗。就诊时右眼最佳矫正视力(BCVA)为20/200,左眼为20/25。双眼眼压均为18 mmHg。裂隙灯检查显示双眼前段无异常发现,包括无细胞和闪光。眼底检查显示右眼介质模糊,呈典型的玻璃丝样外观。B超检查显示玻璃体充满中等回声光斑、玻璃体混浊,并发生了玻璃体后脱离。患者接受了玻璃体活检及标准的25G经睫状体平坦部玻璃体切除术(诊断性和治疗性)。术中发现眼内有严重的弥漫性碎屑和非常强的玻璃体视网膜粘连。玻璃体抽吸物制备的细胞涂片显示无定形无细胞物质,刚果红染色阳性,偏振显微镜下呈苹果绿双折射,符合淀粉样变性的诊断。舌组织的基因评估显示为载脂蛋白AI源性淀粉样变性。术后3个月时双眼BCVA为20/25。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6bc5/7383199/903df3e0bf70/cop-0011-0287-g01.jpg

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