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      A Case of Crystalline Keratopathy in Monoclonal Gammopathy of Undetermined Significance (MGUS)

      case-report

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          Abstract

          A 62-year-old female visited our clinic with progressively decreased vision in both eyes beginning 12 years prior. Idiopathic corneal opacity in all layers of the cornea was found in both eyes. One year later, we performed penetrating keratoplasty on the undiagnosed right eye. During post-surgical follow-up, corneal edema and stromal opacity recurred, and penetrating keratoplasty was performed two more times. The patient's total serum protein level, which had previously been normal, was elevated prior to the final surgery. She was diagnosed with monoclonal gammopathy of undetermined significance. We made a final diagnosis of monoclonal gammopathy-associated crystalline keratopathy after corneal biopsy. Monoclonal gammopathy-associated crystalline keratopathy is difficult to diagnose and may lead to severe visual loss. A systemic work-up, including serologic tests like serum protein or cholesterol levels, is needed in patients with unexplainable corneal opacity.

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          Most cited references13

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          Immunotactoid keratopathy: a clinicopathologic case report and a review of reports of corneal involvement in systemic paraproteinemias.

          Corneal deposits in association with paraproteinemias have been well described in the ophthalmic literature. Recent reports in the renal literature have described immunotactoid deposition associated with glomerulopathy-organized microtubular deposits of IgGkappa that measured 32-50 nm in diameter on renal biopsies. We present a case of corneal immunotactoid deposition in the setting of chronic lymphocytic leukemia and review previous reports of corneal deposition in the setting of systemic paraproteinemia, highlighting the etiology, differential diagnosis, prognosis, and treatment of corneal involvement. We propose the use of the term immunotactoid keratopathy to describe corneal IgGkappa deposits appearing as tubular, electron-dense, crystalloid deposits with a central lucent core on electron microscopy and suggest that these patients undergo directed systemic workup to evaluate for potential etiologies of their systemic paraproteinemia. To illustrate the spectrum of paraprotein deposition in the cornea, and to emphasize the importance of ophthalmic evaluation in the setting of systemic paraproteinemias, we include a case of a 44-year-old man with immunoprotein corneal deposition who was subsequently diagnosed with multiple myeloma.
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            Paraproteinemic crystalline keratopathy.

            Paraproteinemic crystalline keratopathy is an uncommon complication of multiple myeloma and other plasma cell dyscrasias. A case of 16 years' duration was associated with an IgG kappa monoclonal gammopathy and recurrent uveitis. The corneal changes were unusually extensive and distributed throughout all corneal layers. The deposits consisted of diffuse, small, polymorphic aggregates, which were iridescent in the superficial cornea, mat elsewhere, and associated with a diffuse stromal haze; specular microscopy showed additional features. Both corneas were thickened. The deposits failed to stain histochemically, except patchily with Masson Trichrome. There was extensive immunohistochemical labeling for IgG, kappa, and surprisingly, lambda. Ultrastructurally, pleomorphic deposits were found in every corneal cell; paracrystalline deposits with internal banding were seen only in the basal epithelium. Keratocytes and endothelial cells were damaged and reduced in number. Disease recurred in a corneal graft. Three main types of paraproteinemic keratopathy can be distinguished. The deposits probably represent various metabolic products of the monoclonal protein. This is not necessarily a benign condition; hematologic control may be necessary on purely ophthalmological criteria.
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              Corneal crystalline deposits associated with dysproteinemia. Report of two cases and review of the literature.

              Two patients had multiple, fine, polychromatic corneal crystals on ocular examination. Peripheral corneal biopsies and electron microscopic studies demonstrated that the crystals, which were located solely within the cytoplasm of keratocytes, exhibited an internal periodicity of 10 nm and developed within dilated cisternae of rough-surfaced endoplasmic reticulum. Histochemical studies of the corneal crystals confirmed their proteinaceous nature (immunoglobulin crystals). Serum protein analysis showed that both patients had a monoclonal gammopathy with an elevated IgG level and increased K light chains. The associated proteinopathy was unsuspected in both patients until the nature of the crystals was established. Both patients had a neoplastic process; one had a previous diagnosis of Hodgkin's disease, and multiple myeloma developed in the other on follow-up studies. Clinicians should be aware of the possibility of a malignant disorder in patients with corneal crystal-line deposits.
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                Author and article information

                Journal
                Korean J Ophthalmol
                KJO
                Korean Journal of Ophthalmology : KJO
                The Korean Ophthalmological Society
                1011-8942
                2092-9382
                June 2011
                24 May 2011
                : 25
                : 3
                : 202-205
                Affiliations
                Department of Ophthalmology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea.
                Author notes
                Corresponding Author: Jae Chan Kim, MD, PhD. Department of Ophthalmology, Chung-Ang University Yongsan Hospital, #65-207 Hangangno 3-ga, Yongsan-gu, Seoul 140-757, Korea. Tel: 82-2-74-9838, Fax: 82-2-792-6295, jck50ey@ 123456kornet.net
                Article
                10.3341/kjo.2011.25.3.202
                3102825
                21655047
                2cca99d3-297a-44ff-9bdc-9f6458a5d74d
                © 2011 The Korean Ophthalmological Society

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 01 February 2010
                : 07 July 2010
                Categories
                Case Report

                Ophthalmology & Optometry
                corneal opacity,paraproteinemias,crystallins
                Ophthalmology & Optometry
                corneal opacity, paraproteinemias, crystallins

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