Hyperviscosity-Related Retinopathy in Waldenström’s Macroglobulinemia
A 60-year-old man presented with a 2-month history of bilateral blurry vision. The best corrected visual acuity was 10/20 in each eye. Funduscopic examination revealed bilateral retinal-vein dilatation and tortuosity with retinal hemorrhages (Panels A and B show the right and left eyes, respectively). Fluorescein angiography revealed retinal-vein beading patterns with multiple aneurysmal changes and vessel-wall staining (Panels C and D). Serum protein immunofixation assay showed prominent monoclonal bands against the antiserum of IgM and kappa light chains. Bone marrow biopsy revealed infiltration of plasmacytoid lymphocytes (10 to 13%) with expression of CD138 and CD20 markers. After seven rounds of plasmapheresis, visual acuity gradually improved to 20/20. Follow-up funduscopic examination revealed resolution of retinal-vein abnormality. Waldenström’s macroglobulinemia is a lymphoproliferative B-cell disorder characterized by the overproduction of monoclonal IgM. Persons with this disorder can present with the hyperviscosity syndrome, which can manifest as vision changes, cutaneous bleeding, and neurologic symptoms. Vascular abnormalities are not uncommon findings in the hyperviscosity syndrome.