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一名成年非裔美国糖尿病女性的眼眶散发性伯基特淋巴瘤及成人眼眶病例综述

Orbital sporadic Burkitt lymphoma in an adult diabetic African American female and a review of adult orbital cases.

作者信息

Carmody John, Misra Raghunath P, Langford Marlyn P, Byrd William A, Ditta Lauren, Vekovius Bryan, Texada Donald E

机构信息

Department of Ophthalmology.

出版信息

Clin Ophthalmol. 2011;5:509-15. doi: 10.2147/OPTH.S16751. Epub 2011 Apr 20.

Abstract

A case of sporadic Burkitt lymphoma (sBL) presenting with jaw and lid involvement in a diabetic adult African American female and a review of adult orbital Burkitt lymphoma cases are presented. Lid edema, visual loss, ophthalmoparesis, proptosis, and sinusitis progressed over 4 weeks despite antibiotic and steroid treatment. Upper lid biopsy histopathological evaluation and immunophenotyping revealed a homogenous mass of atypical CD10 and CD20-negative B-cells and tingible body macrophages yielding a "starry sky" appearance. Cytogenetic analysis detected a minor variant c-MYC translocation, but no Epstein-Barr virus RNA. Detection of multiple lesions prompted a diagnosis of stage IV disease that totally regressed following radiation and chemotherapy. Review results of the six adult orbital sBL cases support a poor prognosis and a heightened suspicion of variant CD10, CD20 and BCL6 positive sBL in adults presenting with jaw pain and rapidly progressive orbital symptoms, particularly in female, African American, and diabetic patients.

摘要

本文介绍了一例散发型伯基特淋巴瘤(sBL),该病例发生于一名患有糖尿病的成年非裔美国女性,表现为颌骨和眼睑受累,并对成人眼眶伯基特淋巴瘤病例进行了综述。尽管进行了抗生素和类固醇治疗,眼睑水肿、视力丧失、眼球运动麻痹、眼球突出和鼻窦炎在4周内仍不断进展。上睑活检的组织病理学评估和免疫表型分析显示,存在大量均一的非典型CD10和CD20阴性B细胞以及吞噬细胞,呈现“星空”外观。细胞遗传学分析检测到一个微小变异的c-MYC易位,但未检测到爱泼斯坦-巴尔病毒RNA。多处病变的发现提示诊断为IV期疾病,经放疗和化疗后完全消退。对6例成人眼眶sBL病例的综述结果表明,对于出现颌骨疼痛和快速进展的眼眶症状的成人患者,尤其是女性、非裔美国人和糖尿病患者,预后较差,且对变异型CD10、CD20和BCL6阳性sBL的怀疑度更高。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7c8/3090307/66f65563b2c0/opth-5-509f1.jpg

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