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

Abstract

Waldenstrom’s macroglobulinemia (WM) is associated with retinal findings of hyperviscosity such as venous dilation, and findings of immunogammopathy maculopathy such as serous macular detachment. The report describes a case of bilateral serous macular detachment with intraretinal schisis-like fluid in a patient with WM. Enhanced depth imaging OCT revealed a thickened choroid with hyper-reflective accumulations in the RPE layer. The ultra-widefield fundus autofluorescence demonstrated a central area of hyperautofluorescence corresponding to the area of serous macular detachment. Ultra-widefield fluorescein angiography was characteristically silent. Intravitreal bevacizumab therapy resulted in significant reduction in intraretinal fluid, but minimal change in subretinal fluid. Long-term follow-up demonstrated alterations in retinal architecture and improved serous detachments.

Hyperviscosity Retinopathy and Immunogammopathy Maculopahy as New Onset of Multiple Myeloma

“…With time, this layer decreases on the outer retinal surface and a pre-RPE layer of granular hyperreflective material develops. This significant accumulation of material may represent degenerated outer segments, precipitation of immunoglobulin, or a combination of both [ 8 ].…”

“…According to some authors, the slow response of maculopathy to systemic chemotherapy may be due to 3 factors: (1) unknown period that IgM may persist in the subretinal space, either through direct accumulation or osmotic effects; (2) the degree of continued RPE and/or choriocapillaris dysfunction contribute to the pathogenesis [ 8 ]; and (3) long-standing SMD associated with intraretinal cysts. All 3 factors combined would lead to permanent vision loss as a result of RPE and photoreceptor degeneration [ 5 ].…”

Localized retinal degeneration secondary to Waldenström's macroglobulinemia

“…These serous detachments have been variably treated with intravitreal injections of antivascular endothelial growth factor antibodies and dexamethasone implants without much change in the subretinal fluid. [ 11 12 13 ] They only helped in reducing intraretinal fluid and retinal thickness associated with hyperviscosity. We did not offer any active intervention to our patient owing to the extensive atrophic changes.…”

What the Silent Retina Tells You: Serous Retinal Detachment in Waldenström’s Macroglobulinemia

“…This is reflected in the literature by a median IgM level of 5300 mg/dL in the reported cases of WM patients with SMD. 3 – 5 , 7 , 12 – 14 …”

“…The role of local therapy with intravitreal anti-VEGF injections remains unclear as these have shown varying results in the literature. 9 , 11 , 12 In 2 case reports, there was improvement in visual acuity and resolution of subretinal fluid after treatment with intravitreal bevacizumab; however, this was applied concurrently with systemic therapy and plasmapheresis. 9 , 11 The effect of VEGF injections versus the prolonged lowering of IgM levels cannot be distinguished from one another.…”