胰腺结外Rosai-Dorfman病表现为双侧肉芽肿性前葡萄膜炎。
Extranodal Rosai-Dorfman of the Pancreas Presents with Bilateral Granulomatous Anterior Uveitis.
作者信息
Brown A'sha, Branson Sara V, Datoo O'Keefe Ghazala A
机构信息
Emory University School of Medicine, Emory Eye Center, Medical Retinal and Uveitis Fellow, Atlanta, Georgia, USA.
Emory University School of Medicine, Emory Eye Center, Resident, Atlanta, Georgia, USA.
出版信息
Ocul Oncol Pathol. 2019 Jun;5(4):229-233. doi: 10.1159/000493639. Epub 2018 Oct 25.
BACKGROUND/AIMS: To report a case of a 65-year-old female who presented with bilateral anterior granulomatous uveitis, which led to the diagnosis of Rosai-Dorfman Disease (RDD) and the discovery of an associated pancreatic mass.
METHODS
This is a retrospective case report of a single patient.
RESULTS
A 65-year-old African American Female received a full workup for bilateral anterior granulomatous uveitis and was found to have a pancreatic mass on imaging. Biopsy of this mass was diagnostic for RDD, a rare proliferative histiocytic disorder, which classically presents with painless cervical lymphadenopathy, leukocytosis, and hypergammaglobulinemia.
CONCLUSION
Although rare, ocular involvement, including eyelid and orbital masses, and anterior uveitis, can herald the development of other systemic manifestations or RDD and can aid in the diagnosis of this systemic disease.
背景/目的：报告一例65岁女性，其表现为双侧前葡萄膜炎性肉芽肿，最终诊断为罗萨伊-多夫曼病（RDD）并发现伴有胰腺肿块。
方法
这是一例单病例回顾性报告。
结果
一名65岁非裔美国女性因双侧前葡萄膜炎性肉芽肿接受了全面检查，影像学检查发现胰腺有肿块。该肿块活检诊断为RDD，这是一种罕见的增殖性组织细胞疾病，典型表现为无痛性颈部淋巴结病、白细胞增多和高球蛋白血症。
结论
虽然罕见，但眼部受累，包括眼睑和眼眶肿块以及前葡萄膜炎，可能预示着其他全身表现或RDD的发展，并有助于诊断这种全身性疾病。
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