Demirci Hakan, Shields Carol L, Karatza Ekaterine C, Shields Jerry A
Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania, USA.
Ophthalmology. 2008 Sep;115(9):1626-31, 1631.e1-3. doi: 10.1016/j.ophtha.2008.02.004. Epub 2008 Apr 28.
To evaluate the risk for systemic lymphoma (SL) in the patients with orbital lymphoproliferative tumor (OLT).
Observational, retrospective case series.
One hundred sixty consecutive cases with OLT.
Clinical features and treatment method were collected retrospectively. Data from 106 patients without systemic disease at presentation were analyzed for their impact on the main outcome measure using univariate and multivariate regression models.
Occurrence of SL diagnosed based on the 6 monthly systemic evaluation.
Of 106 patients with OLT alone, SL subsequently developed in 16% of patients and 84% patients remained free of SL. Of 17 patients in whom SL developed subsequently, 29% had marginal zone, B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT), 24% had small lymphocytic lymphoma (SLL), 24% had atypical lymphoid hyperplasia (ALH), 6% each had mantle cell, follicular, and diffuse large B-cell lymphoma (DLCL). In these 17 patients, systemic disease appeared after a mean interval of 152 months, and the involved systemic sites were abdominal lymph nodes (LN) in 44% patients, pelvic LN in 40%, and head and neck LN in 31%. Of 17 patients, 53% had the same SL classification with orbital tumor and 47% had a different SL classification. Among 8 patients with different systemic and orbital lymphoma classifications, systemic SLL developed in 4 patients with orbital ALH and in 2 patients with MALT. Two patients with orbital SLL manifested systemic DLCL. Using Kaplan-Meier estimates of 106 OLT patients without systemic involvement, SL developed in 14% at 3 years, in 17% at 5 years, and in 33% at 10 years. Using Kaplan-Meier estimates of 24 patients with bilateral OLT alone, SL developed in 18% at 3 years, in 29% at 5 years, and in 72% at 10 years. In 82 patients with unilateral OLT alone, SL developed in 12% at 3, 5, and 10 years. Multivariate analysis showed that bilateral involvement at presentation was the only significant factor predictive of SL.
In patients with OLT alone at presentation, SL eventually developed in 33% by 10 years in this retrospective case series. Classification of SL can be the same or different from OLT. Development of SL is significantly associated with bilateral involvement.
评估眼眶淋巴增殖性肿瘤（OLT）患者发生系统性淋巴瘤（SL）的风险。
观察性、回顾性病例系列研究。
160例连续的OLT患者。
回顾性收集临床特征和治疗方法。对106例初诊时无全身疾病的患者的数据进行单因素和多因素回归模型分析，以评估其对主要结局指标的影响。
根据每6个月进行的全身评估诊断出的SL的发生情况。
在106例单纯OLT患者中，随后有16%的患者发生了SL，84%的患者未发生SL。在随后发生SL的17例患者中，29%为黏膜相关淋巴组织边缘区B细胞淋巴瘤（MALT），24%为小淋巴细胞淋巴瘤（SLL），24%为非典型淋巴组织增生（ALH），各有6%为套细胞淋巴瘤、滤泡性淋巴瘤和弥漫性大B细胞淋巴瘤（DLCL）。在这17例患者中，全身疾病出现的平均间隔时间为152个月，受累的全身部位中，44%为腹部淋巴结（LN），40%为盆腔LN，31%为头颈部LN。17例患者中，53%的SL分类与眼眶肿瘤相同，47%不同。在8例全身和眼眶淋巴瘤分类不同的患者中，4例眼眶ALH患者和2例MALT患者发生了系统性SLL。2例眼眶SLL患者表现为系统性DLCL。对106例无全身受累的OLT患者进行Kaplan-Meier估计，3年时14%发生SL，5年时17%，10年时33%。对24例仅双侧OLT患者进行Kaplan-Meier估计，3年时18%发生SL，5年时29%，10年时72%。在82例仅单侧OLT患者中，3年、5年和10年时12%发生SL。多因素分析显示，初诊时双侧受累是预测SL的唯一显著因素。
在本回顾性病例系列中，初诊时仅患有OLT的患者，到10年时最终有33%发生了SL。SL的分类可能与OLT相同或不同。SL的发生与双侧受累显著相关。
