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      In vivo confocal microscopic characteristics of crystalline keratopathy in patients with monoclonal gammopathy: Report of two cases

      case-report

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          Abstract

          In this paper, we report two cases of a 62-year-old patient presented with blurred vision and a 45-year-old male diagnosed with multiple myeloma who was referred from the Department of Oncology. Slit-lamp examination, in vivo confocal microscopy (IVCM), systemic work-up and serum protein electrophoresis were obtained. In both patients, slit-lamp findings revealed bilateral diffuse subepithelial and anterior stromal crystals and IVCM showed highly reflective deposits in the corneal epithelium and stroma. The first patient was eventually diagnosed with monoclonal gammopathy of undetermined significance following bone marrow biopsy and systemic evaluation. Unusual corneal deposits may constitute the first sign of monoclonal gammopathies. IVCM may be helpful in showing the crystalline nature of the corneal deposits and guiding the clinician to the diagnosis of gammopathies. Both ophthalmologists and oncologists should be aware that corneal deposits may herald a life-threatening hematologic disease.

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          Most cited references9

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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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            Crystal-storing histiocytosis and crystalline tissue deposition in multiple myeloma.

            Systemic distribution of crystal-storing histiocytes, increasing in number, and widespread crystalline tissue deposition were found in a 75-year-old man with a 5-year history of IgG-kappa-type multiple myeloma associated with corneal opacity and chronic renal failure. Characteristic crystalline inclusions were present not only in myeloma cells but also in cornea, epithelial cells of glomeruli, tubuli, Bowman's capsules, and choroid plexus. Histiocytes had particularly infiltrated the renal interstitium. These inclusions were positive by immunofluorescence for kappa light and gamma heavy chains. By electron microscopy, the inclusions were filled with fine crystalline hexagonal columns, each possessing a core structure. Of various factors generally considered responsible for renal failure in multiple myeloma, marked infiltration of histiocytes and the nephrotoxic effects of light chain appeared most relevant in the present case.
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              Confocal microscopy in multiple myeloma crystalline keratopathy.

              To report confocal microscopy findings of a patient with multiple myeloma crystalline keratopathy and the response to treatment.
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                Author and article information

                Journal
                Indian J Ophthalmol
                Indian J Ophthalmol
                IJO
                Indian Journal of Ophthalmology
                Medknow Publications & Media Pvt Ltd (India )
                0301-4738
                1998-3689
                September 2014
                : 62
                : 9
                : 938-940
                Affiliations
                [1]Department of Ophthalmology, Hacettepe University, School of Medicine, Ankara, Turkey
                [1 ]Department of Hematology, Hacettepe University, School of Medicine, Ankara, Turkey
                [2 ]Department of Pathology, Hacettepe University, School of Medicine, Ankara, Turkey
                Author notes
                Correspondence to: Dr. Murat Irkec, Hacettepe University Department of Ophthalmology, Ankara, Turkey. E-mail: mirkec@ 123456isnet.net.tr
                Article
                IJO-62-938
                10.4103/0301-4738.143933
                4244741
                25370397
                2b1fea25-14a1-4ce0-a8e9-15cbcf7015dd
                Copyright: © Indian Journal of Ophthalmology

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 15 November 2013
                : 06 June 2014
                Categories
                Current Ophthalmology

                Ophthalmology & Optometry
                corneal deposits,in vivo confocal microscopy,monoclonal gammopathy of undetermined significance,multiple myeloma

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