Background: The possibility of hyperviscosity syndrome needs to be considered in cases showing bilateral findings mimicking central retinal vein occlusion. We present our experience with a case of hyperviscosity syndrome due to Waldenström's macroglobulinemia treated with plasmapheresis and chemotherapy, including a discussion on optical coherence tomography (OCT) images of the optic fundus.
Case: A 57-year-old man presented with a bilateral decrease in visual acuity over the past 2-3 months. His previous doctor had suspected central retinal vein occlusion in the right eye, and had referred him to our hospital. At the initial visit, dilatation and tortuosity of bilateral retinal veins was confirmed. OCT images revealed serous retinal detachment and retinal swelling of posterior pole bilaterally. Hyperviscosity syndrome was considered a differential diagnosis because the findings were atypical for central retinal vein occlusion. He was referred to the hematology department of our hospital, where he was diagnosed with Waldenström's macroglobulinemia. He was immediately treated with plasmapheresis and dexamethasone, rituximab and cyclophosphamide (DRC) therapy. Follow-up OCT images to evaluate treatment response showed a gradual, bilateral decrease in the serous retinal detachment and retinal swelling, along with a bilateral decrease in choroidal thickness.
Conclusions: In cases of macular edema accompanied with dilatation and tortuosity of bilateral retinal veins, hyperviscosity syndrome needs to be considered in the differential diagnosis. Follow-up OCT images showed that serous retinal detachment and choroidal thickness decreased proportionally to decreased serum M protein levels.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 121: 923-929, 2017.