Immunogammopathies and Acquired Vitelliform Detachments: A Report of Four Cases

Paraneoplastic Cloudy Vitelliform Submaculopathy in Primary Vitreoretinal Lymphoma

“…More recently, acquired vitelliform lesions, defined as subretinal accretion of clinically yellow, hyper-autofluorescent material above the RPE band and within the macular region not owing to acquired vitelliform macular dystrophies, 26 have been described in association with a variety of conditions, including cuticular drusen, reticular pseudodrusen, serous pigment epithelial detachment, pseudoxanthoma elasticum with angioid streaks, vitreomacular traction, central serous chorioretinopathy, immunogammopathies, and acute exudative paraneoplastic polymorphous vitelliform maculopathy. [26][27][28][29][30][31][32][33][34] We present 3 patients with cloudy subretinal vitelliform maculopathy that showed features distinct from pseudovitelliform macular dystrophy and acquired vitelliform lesions, resolved spontaneously within 3 months of initial presentation, and were ultimately recognized to have underlying primary vitreoretinal lymphoma and/or primary central nervous system lymphoma. The transient nature of this retinopathy suggests a paraneoplastic process.…”

Bevacizumab Therapy and Multimodal Ultrawide-Field Imaging in Immunogammopathy Maculopathy Secondary to Waldenström’s Macroglobulinemia

“…In serous retinal detachment, the physical separation of the RPE from the photoreceptors impairs phagocytosis of the outer segments, leading to an accumulation of fluorophores that appear hyperautofluorescent on fundus autofluorescence. 8,12 The progression of the OCT supports the changes on FAF. Initially, there is a thick irregular border of hyperreflective material on the under surface of the detached retina likely representing degenerating photoreceptor outer segments.…”

“…5–8 The pathogenesis of the maculopathy however remains speculative. It has been shown that other immunogammopathies such as multiple myeloma (which usually has normal serum viscosity) can also produce serous macular detachments.…”

“…It has been shown that other immunogammopathies such as multiple myeloma (which usually has normal serum viscosity) can also produce serous macular detachments. 8 Post-mortem immunofluorescent labeling in a patient with WM has detected IgM within the cystoid spaces of the outer plexiform layer, in the superficial retina and around photoreceptors. 9 IgM has also been detected by immunoelectrophoresis in the subretinal fluid of a patient with Waldenstrom’s.…”

“…The FAF demonstrates hyperautofluorescence in the distribution of the serous detachments, similar to the findings in patients with multiple myeloma and light chain deposition disease. 8 The etiology of the altered FAF is likely multifactorial: autofluorescence occurs due to fluorophores generally found at the level of the RPE with RPE lipofuscin the most studied signal. In serous retinal detachment, the physical separation of the RPE from the photoreceptors impairs phagocytosis of the outer segments, leading to an accumulation of fluorophores that appear hyperautofluorescent on fundus autofluorescence.…”