Carmody John, Misra Raghunath P, Langford Marlyn P, Byrd William A, Ditta Lauren, Vekovius Bryan, Texada Donald E
Department of Ophthalmology.
Clin Ophthalmol. 2011;5:509-15. doi: 10.2147/OPTH.S16751. Epub 2011 Apr 20.
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.
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。
