Hyperviscosity Syndrome-related Retinopathy in a Patient with Waldenström Macroglobulinemia

Purpose: We report a case of retinopathy related to bilateral hyperviscosity syndrome in a patient with Waldenström macroglobulinemia. Case summary: A 77-year-old male presented with reduced visual acuities of both eyes 2 months in duration. Fundus examination revealed extensive flame-shaped retinal hemorrhages and venous congestion. Optical coherence tomography (OCT) evidenced macular edema and OCT angiography (OCTA) indicated damage to the superficial and deep capillary plexi of retina and choriocapillaris and dilated large choroidal vessels. We diagnosed central retinal vein occlusion with macular edema and prescribed intravitreal bevacizumab and posterior subtenon triamcinolone injections, however, the edema did not improve. As retinopathy associated with hematological disease was thus suspected, we referred the patient to our department of hematology and oncology for further assessment. He was diagnosed with Waldenström macroglobulinemia. After initiation of chemotherapy, the retinal hemorrhage in and macular edema of both eyes decreased. After six chemotherapy cycles, the retinal hemorrhages resolved and the macular edema improved in both eyes. OCTA revealed that the choroidal vessel dilation also improved. Conclusions: In elderly patients presenting with central retinal vein occlusions and macular edema of both eyes, it is important to assess whether the retinopathy is associated with a hyperviscosity syndrome linked to a hematological disease.