Department of Ophthalmology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan.
Institute of Clinical Medicine, College of Medicine, National Cheng Kung University, Tainan.
Eur J Ophthalmol. 2022 Jul;32(4):NP109-NP114. doi: 10.1177/11206721211002066. Epub 2021 Mar 15.
To present a 5-year mortal case of Waldenström's macroglobulinemia-related retinopathy and serous macular detachment.
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.
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.
报告一例 5 年死亡的瓦尔登斯特伦巨球蛋白血症相关视网膜病变和浆液性黄斑脱离病例。
一名 63 岁男性,无明显病史,因双眼视力下降 2 个月就诊。眼底检查显示双侧散在视网膜出血、渗出、静脉迂曲和浆液性黄斑脱离。血液学和生化检查显示全血细胞减少,血涂片显示红细胞缗钱状形成。怀疑存在高黏滞综合征,随后通过骨髓活检和血清 IgM 浓度升高诊断为瓦尔登斯特伦巨球蛋白血症。安排了血浆置换和随后的化疗。尽管大多数视网膜异常得到缓解,但他的视力仍无改善,双侧黄斑仍持续脱离。诊断为瓦尔登斯特伦巨球蛋白血症 5 年后,患者死亡。
IgM 对视网膜色素上皮的长期毒性可能会阻碍持续浆液性黄斑脱离的消退,导致其视力无法恢复。因此,早期诊断和通过血浆置换和化疗及时降低血清副蛋白对于预防对患者健康和视力的永久性损害至关重要。