A 65-year-old male presented with complaints of blurred vision OS beginning 3 years ago. He had visited a local hospital where the visual acuity was reported as 6/6 in the right eye and 6/10 in the left. He was diagnosed with branch retinal artery occlusion OS. Two years later, he developed blurry vision in his right eye. He visited another hospital and his vision was 6/60 OD and CF/10 cm OS then. Ophthalmoscopic examination showed retinal vascular occlusion with retinal hemorrhage in the right eye (Fig. 57.1). Fluorescein angiography revealed peripheral retinal vasculitis in both eyes (Fig. 57.2). MR imaging of brain was normal. He was then referred to our hospital. OCT revealed thinning of the inner retinal layer in both eyes (Fig. 57.3). He received panretinal photocoagulation OD and focal laser photocoagulation OS. His visual acuity was CF/20 cm in both eyes 6 months later. A full-field electroretinogram showed decreased amplitudes in both rod and cone functions. The visual fields were severely constricted with a residual central island in both eyes (Fig. 57.4). OCT found severe thinning of the peripapillary retinal nerve fiber layer (RNFL) and macular ganglion cell-inner plexiform layer (GC-IPL) in both eyes (Fig. 57.5). Peripheral blood examination found positive ANA (1:160), TPHA (1:640), elevated ESR (93 mm/h), elevated IgM (>3100 mg/dL), and prolonged aPTT (38.6 s). CRP, IgG/A/E, cardiolipin IgG, lupus anticoagulant, anti-B2-glycoprotein, RF, SSA/SSB, protein C/S, antithrombin III, homocysteine, and PT time were all normal. A tall monoclonal spike in the gamma-globulin region was found by electrophoresis. Immunoelectrophoresis showed an IgM (kappa) monoclonal gammopathy, which was compatible with Waldenström’s macroglobulinemia. The plasma viscosity was 2.42 (1.16–1.56). He received plasmapheresis with fresh frozen plasma five times. A bone marrow biopsy showed nodular and interstitial infiltration of small lymphoid cells, plasmacytoid lymphocytes, and some plasma cells. The plasma cells were predominantly Kappa- or IgM-positive but only scantly with lambda-, IgG-, or IgA-positive. The clinical picture was compatible with lymphoplasmacytic lymphoma, and therefore, the patient received target and chemotherapy for lymphoma. He also received cataract surgery for both eyes. His vision improved to 6/8.6 OD and 6/60 OS 3 years later.
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