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眼眶罗萨伊-多夫曼病的谱系

The spectrum of orbital Rosai-Dorfman disease.

作者信息

Mohadjer Yasaman, Holds John B, Rootman Jack, Wilson Mathew W, Gigantelli James W, Custer Philip L

机构信息

Department of Ophthalmology, Saint Louis University, MO 63141, USA,

出版信息

Ophthalmic Plast Reconstr Surg. 2006 May-Jun;22(3):163-8. doi: 10.1097/01.iop.0000217563.00975.a3.

DOI:10.1097/01.iop.0000217563.00975.a3
PMID:16714922
Abstract

PURPOSE

To describe the spectrum and treatment of orbital Rosai-Dorfman disease and to review previously documented cases.

METHODS

Retrospective, interventional case series of seven patients and literature review.

RESULTS

Each patient with Rosai-Dorfman disease had unique disease expression requiring aggressive therapy, such as chemotherapy, radiation, and/or surgical excision. One systemically aggressive case presented with intraocular and choroidal invasion, a previously unreported finding. Response to therapy was variable and unpredictable.

CONCLUSIONS

Rosai-Dorfman disease, although historically described as benign and self-limiting, may cause significant morbidity and mortality involving multiple organ systems. Available treatment options may not control the disease. Further research and long-term clinical correlation is necessary.

摘要

目的

描述眼眶罗萨伊-多夫曼病的谱系及治疗方法,并回顾既往记录的病例。

方法

对7例患者进行回顾性、干预性病例系列研究及文献综述。

结果

每例罗萨伊-多夫曼病患者都有独特的疾病表现,需要积极治疗,如化疗、放疗和/或手术切除。1例具有全身侵袭性的病例出现了眼内及脉络膜侵犯,这是一个此前未报告过的发现。治疗反应各不相同且不可预测。

结论

罗萨伊-多夫曼病尽管在历史上被描述为良性且自限性疾病,但可能导致涉及多个器官系统的显著发病率和死亡率。现有的治疗选择可能无法控制该病。有必要进行进一步研究和长期临床关联研究。

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