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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.

DOI:10.1159/000493639
PMID:31367582
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6615342/
Abstract

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.

摘要

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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Pancreatic involvement in Erdheim-Chester disease: a case report and review of the literature.胰腺埃勒-狄克森病( Erdheim-Chester disease ):病例报告及文献复习。
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本文引用的文献

1
Review of Rosai-Dorfman Disease: New Insights into the Pathogenesis of This Rare Disorder.罗萨伊-多夫曼病综述:对这种罕见疾病发病机制的新见解
Acta Haematol. 2017;138(1):14-23. doi: 10.1159/000475588. Epub 2017 Jun 15.
2
Sclerouveitis associated with Rosai-Dorfman disease in an HIV-infected patient.一名艾滋病病毒感染患者中与罗萨伊-多夫曼病相关的巩膜葡萄膜炎。
Ocul Immunol Inflamm. 2014 Jun;22(3):236-8. doi: 10.3109/09273948.2013.767914. Epub 2014 Feb 24.
3
Epibulbar rosai-dorfman disease: novel manifestation and treatment.眼球表面罗萨伊-多夫曼病:新的表现及治疗方法
Arch Ophthalmol. 2012 Sep;130(9):1218-20. doi: 10.1001/archophthalmol.2012.12.
4
Rosai-Dorfman disease simulating nodular scleritis and panuveitis.罗萨伊-多夫曼病模拟结节性巩膜炎和全葡萄膜炎。
Arch Ophthalmol. 2011 Apr;129(4):518-20. doi: 10.1001/archophthalmol.2011.56.
5
Atypical ocular presentations of Rosai-Dorfman disease.Rosai-Dorfman病的非典型眼部表现。
Ocul Immunol Inflamm. 2008 Jan-Feb;16(1):9-15. doi: 10.1080/09273940801923911.
6
Spinal epidural Rosai-Dorfman disease preceding by relapsing uveitis: a case report with literature review.复发性葡萄膜炎前驱的脊柱硬膜外罗萨伊-多夫曼病:一例报告并文献复习
Spinal Cord. 2007 Sep;45(9):641-4. doi: 10.1038/sj.sc.3102006. Epub 2007 Jan 16.
7
Uveitic glaucoma and Rosai-Dorfman disease (sinus histiocytosis).葡萄膜炎性青光眼与罗萨伊-多夫曼病(窦性组织细胞增多症)。
Ocul Immunol Inflamm. 2006 Oct;14(5):305-7. doi: 10.1080/09273940600878829.
8
Relapsing uveitis in association with presumed sinus histiocytosis.
Jpn J Ophthalmol. 2006 Sep-Oct;50(5):484-486. doi: 10.1007/s10384-006-0356-7.
9
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