Bilateral central retinal vein occlusion as an initial presentation of Waldenström macroglobulinemia: a case report

Abstract

Background Waldenström macroglobulinemia is a rare hematological malignancy and is the most common diagnosis in patients with hyperviscosity syndrome. Bilateral central retinal vein occlusion as an initial presentation of hyperviscosity syndrome in Waldenström macroglobulinemia is rare. Case presentation A 42-year-old Nepalese male presented with sudden-onset bilateral painless blurring of vision. Fundus examination revealed bilateral, diffusely dilated, tortuous retinal veins and intraretinal deep blot hemorrhages in all four quadrants of the retina in both eyes; features of bilateral central retinal vein occlusion. Serum electrophoresis showed hypoalbuminemia with an immunoglobulin M kappa monoclonal spike. Bone marrow picture and immunohistochemistry analysis were suggestive of lymphoplasmacytic lymphoma. The patient received systemic therapy for Waldenström macroglobulinemia, along with intravitreal bevacizumab. Conclusion Adequate hydration, plasmapheresis, and a combination of bortezomib, dexamethasone, and rituximab regimen as a systemic therapy may represent an ideal choice for patients with hyperviscosity in Waldenström macroglobulinemia.

Waldenström Macroglobulinemia Induced Bilateral Central Retinal Vein Occlusion with Refractory Macular Edema Treated with Systemic Chemotherapy: Case Report

“…Subsequently, intravitreal bevacizumab injection was administered by a monocular trial in one eye, and serous macular detachment and visual acuity significantly improved 7 days after injection [13]. Shrestha et al [14] reported a case of macular edema due to WM treated with intravitreal bevacizumab and systemic therapy (bortezomib, DEX, and rituximab [VDR]). The patient received a total of six VDR regimens, and his vision significantly improved bilaterally to 6/9.…”

“…Therefore macular edema develops into a chronic and refractory condition [15]. Cases reporting improvement in visual acuity after systemic treatment and intravitreal injection [13,14] also show that rapid treatment is advantageous for improving visual acuity, with a case that visited the hospital within 2 weeks of symptom onset [13] and a case that visited the hospital at 5 days after onset [14]. In our case, the delayed start of treatment for more than 1 year can be considered as the cause of the almost unresponsive macular edema even after intravitreal anti-VEGF injection or systemic therapy.…”

Hyperviscosity Syndrome Induced Bilateral Visual and Auditory Impairment in Therapy Resistant Waldenström Macroglobulinemia with MYD88 and CXCR4 Mutations

“…[5][6][7][8] HVS occurs in 10-30% of patients with WM, and WM accounts for nearly 85% of HVS cases. 9,10 In a cohort of 825 newly diagnosed WM patients, 14% developed symptoms consistent with HVS. 11 CXCR4 mutations, found in 30% of patients with WM, are associated with increased likelihood of developing HVS.…”