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Serous Macular Detachments in a Patient With IgM Paraproteinemia: An Optical Coherence Tomography Study
Arch Ophthalmol. 2001;119:911-913.
INTRODUCTION
An unusual maculopathy with macular detachment has been described in patients with immunogammopathies. We observed this type of maculopathy in a patient with type 2 diabetes mellitus and had the opportunity to study the condition with optical coherence tomography. Optical coherence tomography showed not only a neurosensory macular detachment, but also a retinal pigment epithelium (RPE) detachment.
Report of a Case
A 77-year-old man with type 2 diabetes mellitus had reading and driving difficulties. Physical examination revealed poor diabetic control and monoclonal IgM paraproteinemia (IgM level, 46 g/L; reference range, 0.4-2.3 g/L), with 52% plasmocytes in bone marrow aspirate. There was mild anemia (hemoglobin level, 9-11 g/L) and carotid insufficiency, with 50% to 80% stenosis of the left internal carotid artery and no significant abnormalities in other arteries. The patient was normotensive without intake of antihypertensive agents, and there was no evidence of heart or kidney disease. Initially the diagnosis of Waldenström syndrome was made, and the patient was treated accordingly with intermittent plasmapheresis and chlorambucil. Eight months later, the diagnosis was revised to IgM myeloma based on the typical plasma cell appearance of malignant cells in the bone marrow, the presence of a few osteolytic lesions, the absence of hepatosplenomegaly or lymphadenopathy, and progression under chlorambucil treatment. Treatment was changed to 4 to 6 weekly courses of melphalan and methylprednisolone.
Shortly after his first visual complaints, the patient noted further visual loss and was referred to the Department of Ophthalmology, University Hospital Leuven, Leuven, Belgium. Visual acuity was 20/100 OD and 20/50 OS. Biomicroscopy, funduscopy, and fluorescein angiography showed bilateral nucleus sclerosis and retinopathy with scattered hemorrhages, microaneurysms, and leakage from microvascular lesions consistent with mild nonproliferative diabetic retinopathy. The right macula had a central yellow spot. No age-related RPE changes were seen. The left eye had a superomacular RPE defect. There were no cotton wool spots and no venous engorgement.
Two months later, funduscopy showed a macular serous detachment in the right eye and subtle retinal elevation with a central yellow spot in the left eye. Optical coherence tomography of the right eye demonstrated a large neurosensory detachment and an RPE detachment and of the left eye showed a smaller neurosensory detachment and a tiny RPE detachment that corresponded to the yellow macular spot (Figure 1A). An immunogammopathy maculopathy was diagnosed, and acetazolamide treatment was instituted in addition to chemotherapy and plasmapheresis, taking into account that mild leakage was seen in the areas with microaneurysms.
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Figure 1. Optical coherence tomographic scans of the left eye demonstrating early immunogammopathy maculopathy (A) and subsequent expansion of the retinal pigment epithelium and neurosensory detachments (B).
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Despite therapy, the maculopathy progressed, with prominent serous detachments of RPE and overlying retina in both eyes (Figure 1B). In addition to retinal microvascular changes, a fluorescein angiogram showed bilateral macular hypofluorescence due to fluid masking the deep fluorescence (Figure 2). Repeated laser treatment (argon-green) to the microaneurysms was performed. Despite laser therapy, his maculopathy deteriorated in both eyes (Figure 3). One year after diagnosis of paraproteinemia, visual acuity was 20/300 OD and 20/125 OS.
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Figure 2. Early fluorescein photograph of the right eye showing hemorrhages, microaneurysms, and a macular serous detachment with hypofluorescence.
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Figure 3. Color fundus photograph of the right eye 11 months after diagnosis of paraproteinemia showing retinal microvascular lesions and no signs of age-related macular degeneration. The serous retinal pigment epithelium and neurosensory detachment appears inconspicuous but was striking on biomicroscopy and optical coherence tomography.
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Comment
An unusual maculopathy with serous macular detachment might be observed in patients with serum immunogammopathies.1-4 Patients with serum immunogammopathies might show hyperviscosity-associated retinal vascular changes, including venous dilation, flame-shaped or punctate hemorrhages, and cotton-wool spots. These changes are most frequently seen in patients who produce large amounts of monoclonal IgM. Moreover, RPE detachments that leak fluorescein into the subretinal space and that resemble central serous chorioretinopathy have been reported in 3 patients with paraproteinemia.3 The chorioretinopathy in these patients might have been related to their paraproteinemia, type A personality, corticosteroid use, or a combination of these factors.3 Occasionally, serous macular detachments are observed that are unassociated with any fluorescein angiographic evidence of permeability alteration of either the inner or outer blood-retinal barrier.1, 4 Immunofluorescent staining of the retina in a patient with Waldenström macroglobulinemia has shown the presence of immunoglobulin in the inner retina, centrally in the cystoid spaces, and surrounding the photoreceptors. Based on these studies,1-2 it has been postulated that in immunogammopathy maculopathy, extravasation of immunoglobulin occurs primarily in the retina, with subsequent deposition of immunoglobulins in the subneurosensory retinal space, and prolonged serous elevation due to oncotic pressure. Our study with fluorescein angiography and optical coherence tomography supports the hypothesis of transudative macular detachments. Moreover, our optical coherence tomography study demonstrates that the macular detachment is composed of a neurosensory and an RPE detachment, an unreported finding to date.
Plasmapheresis might induce reattachment of the retina.4 However, patients with diabetes and multiple myeloma might respond poorly to treatment and develop chronic serous macular detachment with loss of central vision, as in our patient.4 Diabetes-induced alterations in capillary permeability might contribute to important leakage of serum protein in the retina and transudation in the subretinal space. In these cases, plasmapheresis and chemotherapy might reduce hyperproteinemia and normalize hyperviscosity but be unable to induce resolution of the serous retinal and RPE detachments.
AUTHOR INFORMATION
Corresponding author: Anita M. Leys, MD, PhD, Department of Ophthalmology, University Hospital Leuven, Kapucijnenvoer 33, 3000 Leuven, Belgium.
Anita Leys, MD, PhD;
Peter Vandenberghe, MD, PhD
Leuven, Belgium
REFERENCES
1. Ho AC, Benson WE, Wong J. Unusual immunogammopathy maculopathy. Ophthalmology. 2000;107:1099-1103.
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2. Thomas EL, Olk RJ, Markman M, et al. Irreversible visual loss in Waldenström's macroglobulinemia. Br J Ophthalmol. 1983;67:102-106.
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3. Cohen SM, Kokame GT, Gass JDM. Paraproteinemias associated with serous detachments of the retinal pigment epithelium and neurosensory retina. Retina. 1996;16:467-473.
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4. Gass JDM. Dysproteinemia and serous macular detachment. In: Gass JDM, ed. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment. 4th ed. St Louis, Mo: Mosby–Year Book Inc; 1997:198-1999.
SECTION EDITOR: W. RICHARD GREEN, MD
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