Department of Neuroscience and Pharmacology, Eye Pathology Institute, University of Copenhagen, Denmark.
Acta Ophthalmol. 2013 Mar;91(2):163-9. doi: 10.1111/j.1755-3768.2011.02337.x. Epub 2012 May 2.
To characterize the clinicopathological features of diffuse large B-cell lymphoma (DLBCL) of the ocular adnexal region.
The present series of orbital and adnexal DLBCLs were found by searching the Danish Registry of Pathology between 1980 and 2009. Histological specimens were re-evaluated using a panel of monoclonal antibodies. Clinical files from all patients with confirmed DLBCL were collected.
A total of 34 patients with DLBCL of the ocular adnexal region were identified. Eighteen of the patients were men. The patients had a median age of 78 years (range 35-97 years). Ninety-seven per cent of the patients had unilateral ocular adnexal region involvement, and the orbit (76%) was the most frequently affected site. Nineteen patients (56%) presented with Stage I lymphoma. Of these, 18 were diagnosed with primary lymphoma. Four patients (12%) had Stage II, one patient (3%) had Stage III and ten patients (29%) presented with Stage IV lymphoma. The 5-year overall survival (OS) rate for the whole study group was 20%. The patients with Stage I lymphoma had a significantly better 5-year OS rate (28%) than patients in Stage II-IV (5-year OS rate, 9%). In Cox regression analysis, concordant bone marrow involvement and the International Prognostic Index (IPI) score were prognostic factors for OS.
Diffuse large B-cell lymphoma of the ocular adnexal region is mainly prevalent in elderly patients. Most patients had unilateral orbital involvement. The overall prognosis is poor. Concordant bone marrow involvement and the IPI score were independent prognostic factors for mortality.
描述眼部附属器弥漫性大 B 细胞淋巴瘤（DLBCL）的临床病理特征。
本研究通过检索丹麦病理学会 1980 年至 2009 年的数据库，发现了一系列眼眶和眼部附属器 DLBCL 病例。采用一组单克隆抗体对组织学标本进行重新评估。收集所有确诊为 DLBCL 患者的临床资料。
共发现 34 例眼部附属器 DLBCL 患者。其中 18 例为男性，患者的中位年龄为 78 岁（范围 35-97 岁）。97%的患者为单侧眼部附属器受累，最常受累的部位是眼眶（76%）。19 例（56%）患者为Ⅰ期淋巴瘤，其中 18 例为原发性淋巴瘤。4 例（12%）为Ⅱ期，1 例（3%）为Ⅲ期，10 例（29%）为Ⅳ期。整个研究组的 5 年总生存率（OS）为 20%。Ⅰ期淋巴瘤患者的 5 年 OS 率（28%）明显高于Ⅱ-Ⅳ期患者（5 年 OS 率，9%）。在 Cox 回归分析中，骨髓受累一致和国际预后指数（IPI）评分是 OS 的预后因素。
眼部附属器弥漫性大 B 细胞淋巴瘤主要发生在老年患者中，大多数患者为单侧眼眶受累。总体预后较差。骨髓受累一致和 IPI 评分是死亡率的独立预后因素。
