Bilateral Crystalline Corneal Deposits as First Clinical Manifestation of Monoclonal Gammopathy: A Case Report

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Abstract

Aims

To report the clinical and diagnostic findings of a patient with bilateral corneal deposits caused by an underlying monoclonal gammopathy.

Methods

Slit-lamp biomicroscopy, confocal microscopy and additional serological tests were performed on a 35-year-old man presenting with bilateral crystalline corneal deposits.

Results

The patient was diagnosed as having monoclonal gammopathy based on elevated levels of serum immunoglobulin G. Confocal microscopy showed highly reflective (protein) deposits throughout the entire cornea, with the highest density in the epithelium and anterior stromal keratocytes.

Conclusions

Monoclonal gammopathy, a potential sign of a life-threatening disease, can lead to dense, bilateral corneal deposits. As such changes can occur long before ocular or systemic discomforts appear, an early diagnosis is crucial. Ophthalmologists should be aware of corneal deposits as potential warning signs of monoclonal gammopathy.

Related collections

Most cited references 6

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

Daniel Garibaldi, John Gottsch, Zenaida de la Cruz … (2004)

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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Incidence of corneal crystals in the monoclonal gammopathies.

R A Kyle, Michael W. Bourne, P Greipp … (1989)

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Corneal crystalline deposits associated with dysproteinemia. Report of two cases and review of the literature.

R Font, H Gelender, Laura Barr (1980)

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

Journal ID (nlm-ta): Case Report Ophthalmol

Journal ID (publisher-id): COP

Title: Case Reports in Ophthalmology

Publisher: S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )

ISSN (Electronic): 1663-2699

Publication date Collection: May-Aug 2011

Publication date (Electronic): 26 July 2011

Publication date PMC-release: 26 July 2011

Volume: 2

Issue: 2

Pages: 222-227

Affiliations

[1] ^aDepartment of Ophthalmology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

[2] ^bPrivate Practice, Hamburg, Germany

Author notes

*Dr. Johannes Steinberg, Department of Ophthalmology, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, DE-20246 Hamburg (Germany), Tel. +49 40 7410 53305, E-Mail j.steinberg@ 123456uke.uni-hamburg.de

Article

Publisher ID: cop0002-0222

DOI: 10.1159/000330334

PMC ID: 3150969

PubMed ID: 21829405

SO-VID: 31aad0a2-a502-4a5b-a9c9-7c2ce8407f61

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License ( http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

History

Page count

Figures: 3, References: 7, Pages: 6

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