Survey of Ophthalmology
Volume 50, Issue 1 , Pages 61-80, January 2005

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

  • Daniel C. Garibaldi, MD

      Affiliations

    • The Eye Pathology Laboratory
    • Department of Pathology, the Johns Hopkins Medical Institutions, Baltimore, Maryland, USA
    • ,
  • John Gottsch, MD

      Affiliations

    • Cornea Service, Wilmer Eye Institute
    • ,
  • Zenaida de la Cruz, MS

      Affiliations

    • The Eye Pathology Laboratory
    • Department of Pathology, the Johns Hopkins Medical Institutions, Baltimore, Maryland, USA
    • ,
  • Mark Haas, MD, PhD

      Affiliations

    • Department of Pathology, the Johns Hopkins Medical Institutions, Baltimore, Maryland, USA
    • ,
  • W. Richard Green, MD

      Affiliations

    • The Eye Pathology Laboratory
    • Department of Pathology, the Johns Hopkins Medical Institutions, Baltimore, Maryland, USA
    • Corresponding Author InformationReprint address: W. Richard Green, MD, Eye Pathology Laboratory, Maumenee Building Room 427, Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, MD 21287-9248.

STEFAN SEREGARD AND MILTON BONIUK, EDITORS

Abstract 

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 IgGκ 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 IgGκ 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 myleoma.

Keywords: cornea, deposition, glomerulopathy, immunoprotein, immunotactoid, multiple myeloma, paraprotein

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 The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article. This work was supported in part by the Independent Order of Odd Fellows, Winston-Salem, North Carolina. Hiroyuki Nambu, MD, translated the Japanese articles.

PII: S0039-6257(04)00157-2

doi:10.1016/j.survophthal.2004.10.002

Survey of Ophthalmology
Volume 50, Issue 1 , Pages 61-80, January 2005

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