Dexamethasone implant for immunogammopathy maculopathy associated with IgA multiple myeloma

Abstract

Background:We describe a case where hyperviscosity retinopathy and immunogammopathy maculopathy were the presenting features of IgA multiple myeloma and report the response of maculopathy to intravitreal injection of dexamethasone implants.Case presentation:A 56-year-old man presented at the Department of Ophthalmology with the chief complain of reduced vision for the past 10 days in both eyes. Ophthalmic examination revealed central retinal vein occlusion resembling signs with severe macular edema in both eyes with prominent serous macular detachment. After comprehensive evaluation, an IgA type kappa multiple myeloma was diagnosed complicated with hyperviscosity-associated retinopathy and immunogammopathy maculopathy. Patient was treated with multiple sessions of plasmapheresis, systemic chemotherapy, and finally intravitreal implants of dexamethasone with complete restoration of macular edema and serous macular detachment in both eyes. The visual function and the hyperviscosity-associated retinopathy were partially restored.Conclusion:Ocular manifestation might be the only presenting sign of a life-threatening disease such as IgA multiple myeloma. A high level of suspicion is required to diagnose and treat such cases promptly and effectively.

Multiple Myeloma Presenting as Recalcitrant Macular Edema

“…Intravitreal treatments including dexamethasone or triamcinolone, antivascular endothelial growth factor injections such as bevacizumab, and rituximab have been tried with varied outcomes. [17][18][19] In our case, chemotherapy led to complete resolution of intraretinal and subretinal fluid in the right eye, whereas the left eye had a small amount of residual intraretinal fluid. Vision in the left eye was limited by the subretinal hyperreflective material, loss of the outer retinal bands, and RPE atrophy.…”

Hyperviscosity Retinopathy and Immunogammopathy Maculopahy as New Onset of Multiple Myeloma

“…Classically, fluorescein angiography demonstrates no fluorescein leakage in the macula, and this “angiographically silent” macular detachment distinguishes immunogammopathy maculopathy from other types of serous macular effusion [ 5 , 6 , 7 ]. This indicates that the blood-retinal barriers at the macular area are intact or at least that the disruption of the blood-retinal barriers is not the primary mechanism for subretinal fluid accumulation [ 6 ].…”

“…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 ].…”

Bilaterale subretinale Flüssigkeit und pigmentierte Ablagerungen bei einer jungen Patientin

“…Man vermutet, dass hämorheologische Veränderungen und vaskuläre Turbulenzen zu Gefäßtortuositas, Exsudaten, Mikroaneurysmen, Cotten-Wool-Spots, Blutungen und Sehnervenschwellungen führen [1]. Außerdem kann es, wie hier beschrieben, zu einer Gammopathie-Makulopathie oder Paraproteinämie-Makulopathie mit seröser Makulaabhebung, intraretinalen Zysten und Exsudaten kommen [5].…”