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  • Macular Findings Point to Immunoproliferative Disorder

    By Jean Shaw
    Selected By: Andrew P. Schachat, MD
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    Journal Highlights

    Ophthalmology Retina, March 2020

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    Smith et al. evaluated four patients with neurosensory macular detachment and asymptomatic monoclonal gammop­athy of undetermined significance (MGUS). They found that angiograph­ically silent and treatment-resistant submacular fluid may point to an underlying immunoproliferative subset of MGUS, which they term monoclonal gammopathy of macular significance. In addition, they found that the condition developed into malignant disease in three of the four patients. Given this heightened risk of malignancy, they recommend that patients with neuro­sensory macular detachment not attrib­utable to known causes should undergo serum protein analysis.

    For this retrospective case series, the researchers gathered clinical, laboratory, and imaging findings for four patients with refractory serous macular detachment associated with MGUS. Two of the patients were diagnosed inciden­tally with MGUS by their primary care team while being followed up for chronic submacular fluid; the others were diagnosed after their retina spe­cialist ordered specific laboratory tests for immunogammopathy.

    Of eight eyes, seven demonstrated neurosensory macular detachment with treatment-resistant submacular fluid and vitelliform material. None of the involved eyes showed signs of signifi­cant hyperviscosity retinopathy, and no definite leakage was evident on flu­orescein angiography. All four patients were resistant to treatments aimed at resolving the subretinal fluid, including anti-VEGF drugs, photodynamic thera­py, and topical dorzolamide.

    Three of the four patients under­went malignant transformation (two to multiple myeloma, and one to lymph­oplasmacytic lymphoma). The fourth patient remained free of any malignan­cy eight years after diagnosis.

    This case series illustrates that MGUS may rarely be associated with sub- and intraretinal fluid that is angiographical­ly silent. The authors hypothesize that preexisting chorioretinal disease—and, in some patients, genetic factors—may predispose patients with MGUS to macular fluid accumulation, and they recommend lab evaluation for immu­nogammopathy in patients who have serous macular detachment that cannot be attributed to known causes.

    The original article can be found here.