Macular retinoschisis in a patient with multiple lymphoproliferative malignancies

Q3 Medicine

American Journal of Ophthalmology Case Reports Pub Date : 2025-05-28 DOI:10.1016/j.ajoc.2025.102358

Justine Cheng , Paul Yang , Andy I. Chen , Todd Williams , Christina Flaxel

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Abstract

Purpose

To describe a case of macular retinoschisis in a patient with multiple systemic lymphoproliferative disorders and the challenges of accurate diagnosis of uveal lymphoma.

Observations

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.

Conclusions

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.

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多发性淋巴细胞增生性恶性肿瘤患者的黄斑视网膜裂

目的描述一例多发性全身性淋巴细胞增生性疾病患者的黄斑视网膜裂及葡萄膜淋巴瘤准确诊断的挑战。66岁男性，右眼单侧黄斑视网膜裂伴脉络膜增厚，全身检查有无炎症或肿瘤。患者最终发展为轻度玻璃体炎，并进行了诊断性玻璃体切除术。玻璃体流式细胞术显示克隆扩增与葡萄膜束成熟B细胞淋巴瘤一致。然而，包括骨髓活检在内的重复系统检查显示了两种额外的系统疾病：Waldenstrom巨球蛋白血症（WM）和单克隆B细胞淋巴细胞增多症。基于B细胞基因重排测试，发现脉络膜淋巴瘤不同于系统性淋巴瘤。患者总共有三种淋巴增生性疾病。诊断后不久，左眼也出现脉络膜增厚。患者接受眼眶外束放疗和扎鲁替尼治疗系统性WM和脉络膜淋巴瘤，改善黄斑视网膜裂和脉络膜增厚。结论我们报告一例黄斑视网膜裂合并脉络膜淋巴瘤及多发性全身性淋巴细胞增生性疾病。全身性疾病和葡萄膜淋巴瘤都导致了该患者独特的眼部表现。我们的病例强调了葡萄膜淋巴瘤诊断的挑战以及组织活检、系统检查和密切监测的重要性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

American Journal of Ophthalmology Case Reports Medicine-Ophthalmology

CiteScore

2.40

自引率

0.00%

发文量

513

审稿时长

16 weeks

期刊介绍： The American Journal of Ophthalmology Case Reports is a peer-reviewed, scientific publication that welcomes the submission of original, previously unpublished case report manuscripts directed to ophthalmologists and visual science specialists. The cases shall be challenging and stimulating but shall also be presented in an educational format to engage the readers as if they are working alongside with the caring clinician scientists to manage the patients. Submissions shall be clear, concise, and well-documented reports. Brief reports and case series submissions on specific themes are also very welcome.