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A healthy man in his 60s presented with a 4-month history of blurred vision in his left eye. On examination, his visual acuity was 20/20 OD and 20/40 OS. Intraocular pressures were normal in each eye. Anterior segment and fundus examination results were normal in the right eye. In the left eye, the media were clear, and there were multiple patchy, yellow choroidal lesions with obliteration of the normal choroidal vasculature throughout the entire fundus (Figure 1A). There was no sign of vitritis or retinitis and no history of previous trauma, uveitis, sarcoidosis, or cancer. Ocular ultrasonography revealed diffuse ciliochoroidal thickening measuring 2.9 mm in elevation. Optical coherence tomography disclosed generalized, undulating choroidal infiltration in a seasick pattern, so to speak, given its resemblance to a stormy ocean surface, with overlying draping of the retina in the foveal region (Figure 1B).
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Choroidal mucosa-associated lymphoid tissue (MALT) lymphoma with plasmacytic differentiation and associated IgM paraproteinemia
C. Complete serum immunoglobulin level tests, DNA molecular analysis, and bone marrow biopsy
The findings on the relook at the anterior segment were suggestive of a choroidal lymphoid process (Figure 2A). The morphology found on incisional biopsy with immunohistochemistry (Figure 2B) was compatible with mature small B-cell lymphoma, including extranodal marginal-zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), with prominent plasmacytic differentiation or lymphoplasmacytic lymphoma.
In this case, systemic oncologic evaluation (choice C) uncovered underlying IgM paraproteinemia (IgM paraprotein level, 280 mg/dL; normal range, 45-250 mg/dL; to convert to grams per liter, multiply by 0.01) without systemic infiltration elsewhere; IgM paraproteinemia is a monoclonal gammopathy that encompasses a group of disorders in which abnormal proteins (paraproteins) are detected in blood. This uncommon condition affects 3.5% of individuals older than 50 years1 and is a nonspecific hematological phenomenon that accounts for 10% to 20% of all monoclonal gammopathies. The excess of IgM protein usually indicates the presence of underlying hematological conditions, such as monoclonal gammopathy of undetermined significance, Waldenström macroglobulinemia, multiple myeloma, amyloidosis, and indolent lymphomas such as MALT.1,2 Elevated serum levels of IgM can result in hyperviscosity syndrome, leading to vascular compromise. Ophthalmic manifestations of monoclonal gammopathies are rare and include immunoglobulin deposition as conjunctival or corneal crystals, choroidal infiltration with secondary subretinal fluid (maculopathy), and tumor formation as uveal plasmacytoma.3- 5
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Corresponding Author: Carol L. Shields, MD, Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, 840 Walnut St, Ste 1440, Philadelphia, PA 19107 (email@example.com).
Published Online: May 21, 2020. doi:10.1001/jamaophthalmol.2020.0452
Conflict of Interest Disclosures: Dr Shields reports support from the Eye Tumor Research Foundation. No other disclosures were reported.
Additional Contributions: We thank Tatyana Milman, MD, the Wills Eye Hospital Pathology Department for histopathology consultation in this case. She was not compensated. We also thank the patient for granting permission to publish this information.
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