May 31, 2019 Conference


Novel retina findings in paraproteinemias in a veteran population

Return to Session

Dr. Lauren Wright, BMC (Presenter)
Dr. Steven Ness
Dr. Vasiliki Poulaki

Purpose:  Retinal findings in lymphoplasmacytic and myeloid disorders are not well characterized. The purpose of this study was to describe retinal manifestations in paraprotein-related diseases. We present a series of cases involving retinal changes secondary to lymphoplasmacytic lymphoma, monoclonal gammopathy of undetermined significance (MGUS), and multiple myeloma. Methods: Medical records were reviewed from 4 patients treated from 2009-2018 at the Boston VA healthcare system in Boston, VA. Dilated ocular exam findings (DFE) and imaging studies were assessed. Case 1: 57 yo male with dyspnea, renal failure, and severe anemia presented with diffuse cotton wool spots and retinal hemorrhages. Sub-retinal and sub-retinal pigment epithelium (RPE) deposits were present along the arcades and macula. Increased serum kappa LC were present with associated glomerulosclerosis. The patient was diagnosed with B-cell lymphoma and concurrent LC disease with ocular involvement. He maintained good visual acuity with stable diffuse RPE deposits throughout the fundus bilaterally. Case 2: 55 yo male presented with diffuse bilateral cotton wool spots. FA showed extensive nonperfusion in the setting of monoclonal gammopathy of undetermined significance (MGUS) with elevated serum IgA and kappa LC. He was referred to hematology for bone marrow (BM) biopsy. Case 3: 64 yo male with hx of MGUS presented with unilateral vitreous hemorrhage. His clinical course was marked by progressive retinal ischemia bilaterally. FA showed macular ischemia with hyperfluorescence and late leakage. He was treated with intravitreal anti-VEGF and laser. Serum studies showed increased lambda LC and a diagnosis of myeloma was made. Case 4: 69 yo male referred for maculopathy evaluation. DFE was significant for scattered RPE changes and tortuous vessels. FA showed numerous areas of hyperfluorescence that leaked over time without ischemia. OCT showed central RPE level hyperreflectivity, mild SRF, and RPE detachments. BM biopsy showed plasma cell dyscrasia with lambda restricted plasma cells. Conclusion: We present a case series of 4 patients with light chain paraprotein disease with ocular manifestations. Common ocular manifestations among our patients include severe ischemic retinopathy, intra- or sub-retinal fluid, and RPE level deposition disease with variable effects on acuity. Findings were identified  in the absence of serum hyperviscosity. Larger cases series are needed to better delineate the spectrum of disease and pathomechanism of intraocular findings of these disorders.