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Stephen J Smith, Mark W Johnson; Maculopathy in Patients with Monoclonal Gammopathy of Undetermined Significance. Invest. Ophthalmol. Vis. Sci. 2014;55(13):5943.
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To describe the retinal findings, laboratory results, and response to treatment of patients with monoclonal gammopathy of undetermined significance (MGUS) presenting with exudative maculopathy.
We retrospectively reviewed the medical and ocular histories, ophthalmologic examinations, retinal imaging, ocular course, and laboratory findings in 3 patients with exudative maculopathy associated with MGUS.
All three patients (aged 56-78 years) demonstrated treatment resistant sub-macular fluid with vitelliform deposits. No patient demonstrated signs of hyperviscosity retinopathy. Fluorescein angiography showed no definite leakage in any involved eye. Laboratory evaluation revealed IgG kappa MGUS in 2 patients and IgA MGUS in the third patient. Two patients had MGUS features consistent with a higher risk of malignant transformation (elevated free light chain ratio in 1 patient and IgA MGUS in another). Two patients were resistant to all therapy, including anti-VEGF injections, photodynamic therapy (PDT), topical dorzolamide, and/or systemic eplerenone. The third patient responded to PDT; however, laboratory evaluation revealed decreasing serum IgA over the same time interval. With follow-up ranging from 7 to 38 months, none of the patients have exhibited malignant transformation warranting systemic treatment.
Exudative maculopathy unresponsive to conventional treatment may suggest an underlying hematologic condition. Given the propensity for MGUS to transform into malignant disease with time, serum protein analysis may have particular importance in patients presenting with atypical or treatment resistant serous macular detachment.
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