CLINICAL AND IN VIVO CONFOCAL MICROSCOPY FINDINGS IN MGUS - A CASE REPORT

Alfonso Strianese, Valentino de Ruvo, Chiara Quisisana, Francesco Pozzo Giuffrida, Saverio Luccarelli, Francesco Viola, Luca Mario Rossetti, Paolo Fogagnolo
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Abstract

Purpose The purpose of this study was to describe a case of paraproteinemic keratopathy (PPK) in a patient with monoclonal gammopathy of undetermined significance (MGUS) Methods A 56 years old man was assessed using a slit lamp and in vivo confocal microscopy (IVCM) with Heidelberg HRT Rostock II. Results The patient showed bilateral diffuse, symmetric, non-polychromatic, punctiform crystals in all layers of the cornea. The remaining part of the ophthalmic clinical assessment was normal. Esthesiometry was normal in all quadrants. Anterior segment OCT confirmed diffused hyperreflectivity of the epithelium and corneal stroma. IVCM was then executed, showing polymorphic (granular, needle-like, stellate) hyperreflective crystals in all layers of corneal epithelium and anterior stroma, subbasal plexus nerves beading, cytoplasmatic hyperreflectivity of the keratocytes of the posterior stroma without any endothelial involvement After considering the age, health status, and ocular signs the primary entities to be investigated were adult benign cystinosis and paraproteinemic disorder. All the diagnostic tests were negative except for a monoclonal component IgG/k of 1.37 d/dl. A hematological examination was then performed with the resulting diagnosis of MGUS. In this paper, we analyzed 11 articles about IVCM in PPK in MGUS and other paraproteinemias highlighting IVCM features compatible with our findings allowing us to generalize a first diagnostic IVCM pattern for PPK. Conclusions In this case report we suggest the IVCM diagnostic patterns that may be used to identify PPK without corneal biopsy.
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临床和体内共聚焦显微镜发现的mgus - 1例报告
目的本研究的目的是描述一例伴有未确定意义单克隆伽玛病(MGUS)的副蛋白血症性角膜病变(PPK)。方法采用裂隙灯和体内共聚焦显微镜(IVCM)对56岁男性进行评估。结果患者双侧角膜各层均可见弥漫性、对称、非多色、点状晶体。其余部分眼科临床评价正常。所有象限的美感均正常。前段OCT证实上皮和角膜间质弥漫性高反射率。然后进行IVCM,在角膜上皮和前基质的所有层中显示多形态(颗粒状,针状,星状)高反射晶体,基底下丛神经束状,后基质角质细胞的细胞质高反射率,没有任何内皮受损伤。考虑到年龄,健康状况和眼部体征,主要要调查的实体是成人良性胱氨酸病和蛋白副蛋白血症。除单克隆成分IgG/k为1.37 d/dl外,所有诊断试验均为阴性。然后进行血液学检查,诊断为MGUS。在本文中,我们分析了11篇关于IVCM在MGUS和其他副蛋白血症中的PPK的文章,强调IVCM的特征与我们的发现相一致,使我们能够概括出PPK的第一个诊断IVCM模式。结论:在本病例报告中,我们建议IVCM诊断模式可用于识别PPK而无需角膜活检。
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