Serous Macular Detachment in Waldenström Macroglobulinemia: A Report of 4 Cases

We read with great interest the article by Baker and associates. These authors examined the effects of Waldenström disease on 4 patients with serous macular detachment. Immunoglobulin M was identified in the subretinal space and all retinal layers in affected patients, and the accumulation of immunoglobulin M in the subretinal space can lead to sustained osmotic serous macular detachment through fluid transudation from the retinal vessels.

Our experience is based on the observation of a 67-year-old white man with a history of uncompensated type 2 diabetes. His visual acuity was 20/63 in the right eye and 20/40 in the left eye. Serum protein electrophoresis, serum immunofixation, and bone marrow biopsy results confirmed the diagnosis of Waldenström disease. Spectral-domain optical coherence tomography showed bilateral serous macular detachment and cystoid spaces in the external nuclear layer with an intact outer retina.

Dynamic fluorescein angiography revealed a silent fovea, bilateral leakage from microaneurysms along the vascular arcade, midperipheral venous staining and leakage, areas of capillary nonperfusion located nasally to the optic nerve head, and a significant breakdown of the blood-retinal barrier around the optic nerve head and in the mid periphery. Indocyanine green angiography showed a masking effect of the subretinal fluid and floaters in the late phases; no leakage or staining from incompetent retinal pigment epithelium was observed.

Based on the results from the spectral-domain optical coherence tomography, Baker and associates proposed the disruption of the outer retina as a track for intraretinal immunoglobulin M and fluid to enter the subretinal space. However, in our patient, no disruption of the outer retina and external limiting membrane was identified on the spectral-domain optical coherence tomography volume scans obtained during any visit; this finding is not consistent with the pathogenic assumption previously discussed.

Furthermore, Patient four exhibited bilateral cystoid macular edema, as determined using late fluorescein angiography. Consistent with the description of Gass, we observed a silent fovea using fluorescein angiography.

In conclusion, the precise mechanism underlying the macular alterations of the outer retina in Waldenström disease remains poorly understood. Thus, we assert that reports of cases, such as that of Baker and associates, may provide insight into this rare condition.

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Jan 9, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on Serous Macular Detachment in Waldenström Macroglobulinemia: A Report of 4 Cases

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