Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania.
Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania.
Ophthalmology. 2014 Jan;121(1):342-351. doi: 10.1016/j.ophtha.2013.06.046. Epub 2013 Aug 23.
To describe the clinical and histopathologic features of choroidal lymphoma (CL) and its association with systemic lymphoma.
Observational case series.
Fifty-nine patients (73 eyes) with CL.
Retrospective chart review.
Clinical features, histopathology, and systemic lymphoma.
Of 59 patients with CL, systemic lymphoma was absent in 41 (primary CL; 69%) and present in 18 (secondary CL; 31%). Of 18 patients with systemic lymphoma at presentation, 14 (78%) had known systemic lymphoma and 4 (22%) were diagnosed with systemic lymphoma shortly after presentation. The most common types of systemic lymphoma in patients with secondary CL were diffuse large cell lymphoma (n = 5, 28%), chronic lymphocytic leukemia/lymphoma (n = 4, 22%), multiple myeloma (n = 2, 11%), Waldenstrom's macroglobulinemia (n = 2, 11%), extranodal marginal zone lymphoma (n = 2, 11%), plasmablastic lymphoma (n = 1, 6%), and unspecified non-Hodgkin lymphoma (n = 2, 11%). Compared with patients with primary CL, patients with secondary CL had a shorter mean duration of ocular symptoms (5 vs. 17 months), had less often received steroids before referral (11% vs. 46%), and were more likely to have bilateral ocular involvement (33% vs. 20%). Eyes with secondary CL had a higher rate of poor vision (≤20/200) (46% vs. 12%), iris (20% vs. 4%) or ciliary body (30% vs. 8%) lymphoma, episcleral vascular congestion (40% vs. 16%), anterior chamber reaction (30% vs. 14%), hyphema (15% vs. 0%), vitreous cellular infiltration (30% vs. 4%), and severe media haziness (20% vs. 0%). Secondary CL was morphologically more high grade (50% diffuse large cell lymphoma) compared with primary CL (37% low-grade non-Hodgkin lymphoma, 27% extranodal marginal zone lymphoma). None of the 33 patients with primary CL and subsequent follow-up developed systemic lymphoma during a mean follow-up of 50 months (median, 35 months; range, 2-231 months).
Secondary CL is morphologically high grade and associated with more severe ocular findings. Patients with CL and no known systemic lymphoma at presentation should undergo systemic evaluation to rule out the possibility of undiagnosed concurrent systemic lymphoma. However, none of the patients with primary CL in our study had late development of systemic lymphoma.
描述脉络膜淋巴瘤（CL）的临床和组织病理学特征及其与全身淋巴瘤的关系。
观察性病例系列。
59 例（73 只眼）CL 患者。
回顾性病历审查。
临床特征、组织病理学和全身淋巴瘤。
59 例 CL 患者中，41 例（原发性 CL；69%）无全身淋巴瘤，18 例（继发性 CL；31%）有全身淋巴瘤。18 例有全身淋巴瘤的患者中，14 例（78%）已知有全身淋巴瘤，4 例（22%）在就诊后不久被诊断为全身淋巴瘤。继发性 CL 患者中最常见的全身淋巴瘤类型为弥漫性大细胞淋巴瘤（n=5，28%）、慢性淋巴细胞白血病/淋巴瘤（n=4，22%）、多发性骨髓瘤（n=2，11%）、瓦尔登斯特伦巨球蛋白血症（n=2，11%）、结外边缘区淋巴瘤（n=2，11%）、浆母细胞淋巴瘤（n=1，6%）和未特指的非霍奇金淋巴瘤（n=2，11%）。与原发性 CL 患者相比，继发性 CL 患者的眼部症状平均持续时间较短（5 个月 vs. 17 个月），转诊前接受类固醇治疗的比例较低（11% vs. 46%），双眼受累的可能性更大（33% vs. 20%）。继发性 CL 眼视力较差（≤20/200）的比例较高（46% vs. 12%），虹膜（20% vs. 4%）或睫状体（30% vs. 8%）受累，巩膜血管充血（40% vs. 16%）、前房反应（30% vs. 14%）、前房积血（15% vs. 0%）、玻璃体细胞浸润（30% vs. 4%）和严重的介质混浊（20% vs. 0%）。与原发性 CL（37%低度非霍奇金淋巴瘤，27%结外边缘区淋巴瘤）相比，继发性 CL 的形态学分级更高（50%弥漫性大细胞淋巴瘤）。在平均 50 个月（中位数 35 个月；范围 2-231 个月）的随访中，没有 33 例原发性 CL 患者随后发展为全身淋巴瘤。
继发性 CL 形态学分级较高，眼部表现更严重。就诊时无全身淋巴瘤的 CL 患者应进行全身评估，以排除同时存在未确诊的全身淋巴瘤的可能性。然而，在我们的研究中，没有原发性 CL 患者在后期发展为全身淋巴瘤。
