Paraproteinemic Keratopathy
| Autor: | Carrie Zaslow Simon, Ralph C. Eagle, David S. Chu, Annie Steiner, Tatyana Milman, Andrew Allen Kao, Frederick A. Jakobiec, Ira J Udell, Carolyn Shih, Anthony J. Aldave, Matthew Gorski |
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| Rok vydání: | 2015 |
| Předmět: |
Systemic disease
Paraproteinemia Pathology medicine.medical_specialty Visual acuity Interstitial keratitis business.industry Waldenstrom macroglobulinemia medicine.disease eye diseases Ophthalmology Smoldering Plasma Cell Myeloma medicine.anatomical_structure Cornea medicine sense organs medicine.symptom business Monoclonal gammopathy of undetermined significance |
| Zdroj: | Ophthalmology. 122:1748-1756 |
| ISSN: | 0161-6420 |
| DOI: | 10.1016/j.ophtha.2015.05.029 |
| Popis: | Purpose To describe 7 patients with paraproteinemic keratopathy and to highlight the clinical and pathologic diversity of this rare entity and the importance of timely, systemic evaluation. Design Retrospective, multicenter collaborative case series. Participants Seven patients with paraproteinemic keratopathy. Methods Clinical and pathologic records were reviewed to identify patients with well-documented corneal immunoglobulin deposits. Detailed ophthalmologic and medical histories were assembled. In 6 patients, corneal tissue was evaluated histochemically and immunohistochemically; in selected cases, corneal tissue was evaluated by in situ hybridization and ultrastructurally. Main Outcome Measures Visual acuity and anterior segment examination at presentation and follow-up; local therapy; systemic diagnosis and management; and histopathologic, immunohistochemical, in situ hybridization, and ultrastructural findings. Results Seven patients were identified with corneal immunoglobulin deposition. In addition to previously reported crystalline, nummular, patch-like, and lattice-like corneal opacities, prominent corneal vascularization was present in 2 patients mimicking interstitial keratitis and limbal stem cell deficiency. All patients had evidence of paraproteinemia in a setting of monoclonal gammopathy of undetermined significance, smoldering plasma cell myeloma, or Waldenstrom macroglobulinemia. Corneal findings were the first manifestation of systemic disease in 4 patients, and the diagnosis was not suspected in 3 of these patients. Pathologic evaluation of biopsied corneal and conjunctival tissues demonstrated immunoglobulin deposits. Previously unreported ultrastructural patterns in the cornea were noted: large scroll-like immunotactoid deposits, immune complex-like deposits, and randomly arranged fibrils morphologically intermediate between amyloid and immunotactoid deposits. Surgical intervention to improve vision was performed in 4 patients, with recurrence of deposits in 3 patients. Three patients underwent systemic therapy with diminution of the deposits and improvement in vision in 1 patient. Conclusions The clinical and pathologic expressions of corneal immunoglobulin deposits are protean and present a diagnostic challenge. Early recognition of this rare entity is important to address the potentially serious associated systemic disease. |
| Databáze: | OpenAIRE |
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