Researchers in Brazil presented a case report in the journal Case Reports in Ophthalmology of a patient with multiple myeloma (MM) who developed immunogammopathy maculopathy and hyperviscosity syndrome (HVS)-related retinopathy.
The patient was described as a male of 50 years of age who was otherwise healthy, without known systemic or ocular diseases. Immunogammopathy maculopathy and hyperviscosity retinopathy were identified as presenting features of new-onset MM in this patient who had experienced 2 months of visual changes in each eye without pain, including a sudden loss of visual acuity; visual acuity in the left eye was 20/63 and in the right eye was at the level of counting fingers at 1 meter.
On ocular examination, the patient appeared to have features resembling central retinal vein occlusion (CRVO). Macular edema, serous macular detachment, and outer retinal hyperreflective material were found through optical coherence tomography. Engorged, tortuous veins and other changes were seen with fluorescein angiography.
A systemic investigation was undertaken because of this patient’s atypical presentation. His blood pressure was 110/68 mm Hg, and he had anemia, with a hemoglobin level of 5.8 g/dL. He also had a high partial thromboplastin time, high international normalized ratio, hypercalcemia, and renal insufficiency.
A monoclonal protein component with a peak of 7.0 g/dL was identified through serum protein electrophoresis, which was determined by immunofixation to be immunoglobulin M (IgM) kappa. Further analyses revealed lytic bone lesions, with 55% infiltration by plasma cells found in a bone marrow biopsy.
HVS and hypercalcemia were treated by plasmapheresis and zoledronate, respectively. The patient’s MM was treated with bortezomib, cyclophosphamide, and dexamethasone (VCD). With 6 months of VCD therapy, visual acuity had become 20/100 in the left eye and 20/32 in the right eye. Improvement was seen with regard to intraretinal hemorrhages on fundoscopic evaluation. Serous macular detachment remained, and the patient was treated with intravitreal triamcinolone acetonide in the left eye. Visual acuity, serous macular detachment, and hyperreflective deposition in both eyes had not changed 1 month later.
A year later, the patient’s visual acuity had become 20/200 in the left eye and 20/40 in the right eye. He had achieved a partial response and was in his sixth cycle of chemotherapy. He had also become a candidate for autologous stem cell transplantation.
The authors noted that HVS is rare with MM, showing an incidence of 2 to 6% of cases. Serous macular detachment is considered an immunogammopathy maculopathy that has been seen with IgM MM and more often with Waldenström macroglobulinemia.
In this case, the authors wrote, there was a reduction in intraretinal fluid obtained, while serous macular detachment remained. “Thus, HVS-retinopathy improves after plasmapheresis and systemic chemotherapy, but there are no known effective treatments for immunogammopathy maculopathy,” the authors concluded in their report.
Ferreira Santos de Cruz N, Milhomens Filho JAP, Ferraro DMN, Polizelli MU, de Moraes Ambrogini NSB. Hyperviscosity retinopathy and immunogammopathy maculopahy as new onset of multiple myeloma. Case Rep Ophthalmol. 2021;12(2):578-584. doi:10.1159/000514695