Cheng Justine, Yang Paul, Chen Andy I, Williams Todd, Flaxel Christina
Department of Ophthalmology and Visual Sciences, University of Illinois Chicago, Chicago, IL, USA.
Casey Eye Institute, Oregon Health & Science University, Portland, OR, USA.
Am J Ophthalmol Case Rep. 2025 May 28;39:102358. doi: 10.1016/j.ajoc.2025.102358. eCollection 2025 Sep.
To describe a case of macular retinoschisis in a patient with multiple systemic lymphoproliferative disorders and the challenges of accurate diagnosis of uveal lymphoma.
A 66-year-old male presented with unilateral macular retinoschisis and choroidal thickening in the right eye had an unrevealing systemic work-up for inflammatory or neoplastic processes. The patient eventually developed mild vitritis and a diagnostic vitrectomy was performed. Flow cytometry of the vitreous showed clonal expansion consistent with a mature B cell lymphoma of the uveal tract. Repeat systemic work-up including bone marrow biopsy, however, revealed two additional systemic conditions: Waldenstrom macroglobulinemia (WM) and monoclonal B cell lymphocytosis. Based on B cell gene rearrangement testing, the choroidal lymphoma was found to be distinct from the systemic lymphoma. In total, the patient had three lymphoproliferative disorders. Soon after the diagnosis, the left eye also developed choroidal thickening. The patient received orbital external beam radiation and zanubrutinib to treat the systemic WM and the choroidal lymphoma with improvement of macular retinoschisis and choroidal thickening.
We present a case of macular retinoschisis in a patient with choroidal lymphoma and multiple systemic lymphoproliferative disorders. Both the systemic disease and the uveal lymphoma contributed to the unique ocular findings in this patient. Our case highlights the challenges of uveal lymphoma diagnosis and the importance of tissue biopsy, systemic work-up, and close monitoring.
描述一例患有多种系统性淋巴增殖性疾病患者的黄斑视网膜劈裂病例，以及准确诊断葡萄膜淋巴瘤所面临的挑战。
一名66岁男性，右眼出现单侧黄斑视网膜劈裂和脉络膜增厚，针对炎症或肿瘤性病变进行的全身检查未发现异常。患者最终出现轻度玻璃体炎，并进行了诊断性玻璃体切除术。玻璃体的流式细胞术显示克隆性扩增，与葡萄膜成熟B细胞淋巴瘤一致。然而，包括骨髓活检在内的重复全身检查发现了另外两种全身性疾病：华氏巨球蛋白血症（WM）和单克隆B细胞淋巴细胞增多症。基于B细胞基因重排检测，发现脉络膜淋巴瘤与系统性淋巴瘤不同。该患者总共患有三种淋巴增殖性疾病。诊断后不久，左眼也出现了脉络膜增厚。患者接受了眼眶外照射和泽布替尼治疗系统性WM和脉络膜淋巴瘤，黄斑视网膜劈裂和脉络膜增厚情况有所改善。
我们报告了一例患有脉络膜淋巴瘤和多种系统性淋巴增殖性疾病患者的黄斑视网膜劈裂病例。全身性疾病和葡萄膜淋巴瘤均导致了该患者独特的眼部表现。我们的病例突出了葡萄膜淋巴瘤诊断的挑战以及组织活检、全身检查和密切监测的重要性。
