Multimodal imaging of bilateral immunogammopathy maculopathy associated to Waldenström’s macroglobulinemia

Aim: Our aim is to report a case with bilateral Waldenström’s macroglobulinemia (WM) associated maculopathy, assessed with multimodal imaging including swept source optical coherence tomography (SS-OCT) and OCT-Angiography (OCT-A). Methods: Observational case report. Case presentation: A 61-year-old diabetic woman with history of treated WM currently in remission, presented with progressive bilateral visual loss. Best-corrected visual acuity was 20/100 in the right eye (RE) and 20/200 in the left eye (LE). Fundus examination showed bilateral microaneurysms and retinal punctuate hemorrhages and a large macular serous detachment in the LE. There was no retinal ischemia on FA nor macular dye leakage. SS-OCT showed a significant schisis-like intraretinal fluid accumulation in the RE and a large prominent macular detachment with significant subretinal fluid accumulation in the LE. Retinal and choriocapillaris vascular densities were normal on OCT-A. Conclusion: Our case illustrated characteristic multimodal imaging findings in WM associated maculopathy such as schisis-like intraretinal fluid accumulation and angiographically silent serous macular detachment. OCT-A could non-invasively analyze macular vascular densities layer-by-layer, without noticing any vascular anomaly.

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Hyperviscosity-related retinopathy and serous macular detachment in Waldenström’s macroglobulinemia: A mortal case in 5 years

European Journal of Ophthalmology ◽

10.1177/11206721211002066 ◽

2021 ◽

pp. 112067212110020

Author(s):

Chun-Chieh Lai ◽

Chun-Hsiang Chang

Keyword(s):

Pigment Epithelium ◽

Retinal Pigment ◽

Waldenström’S Macroglobulinemia ◽

High Concentration ◽

Serous Macular Detachment ◽

Macular Detachment ◽

Waldenstrom's Macroglobulinemia ◽

Waldenstrom’S Macroglobulinemia ◽

Waldenström's Macroglobulinemia

Purpose: To present a 5-year mortal case of Waldenström’s macroglobulinemia-related retinopathy and serous macular detachment. Case report: A 63-year-old man, with unremarkable medical history, presented with bilateral decreased vision for 2 months. Fundus examination revealed bilateral scattered retinal hemorrhages, exudates, venous tortuosity, and serous macular detachment. Hematologic and biochemistry profiles showed pancytopenia with blood smear demonstrating erythrocyte rouleaux formation. Hyperviscosity syndrome was suspected and later Waldenström’s macroglobulinemia was diagnosed by bone marrow biopsy and high concentration of serum IgM. Plasmapheresis and subsequent chemotherapy were arranged. In spite of resolution of most retinal abnormalities, his visual acuity still showed no improvement with a persistent bilateral macular detachment. The patient then died 5 years after the diagnosis of Waldenström’s macroglobulinemia. Conclusions: Long-term toxicity of IgM to the retinal pigment epithelium may impede the resolution of the persistent serous macular detachment, resulting in an inability of recovery in his vision. Therefore, early diagnosis and timely reduction of serum paraproteins by plasmapheresis and chemotherapy is critical for preventing permanent damages to patients’ health and vision.

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