IgG4 阳性眼附属器边缘区 B 细胞淋巴瘤患者的临床特征和治疗结果。
Clinical characteristics and treatment outcomes of patients with IgG4-positive ocular adnexal marginal zone B-cell lymphoma.
机构信息
Department of Ophthalmology, West China Hospital, Sichuan University, No. 37 Guoxue Xiang, Wuhou District, Chengdu, Sichuan Province, China.
Department of Hematology, West China Hospital, Sichuan University, No. 37 Guoxue Xiang, Wuhou District, Chengdu, Sichuan Province, China.
出版信息
Indian J Ophthalmol. 2024 Nov 1;72(Suppl 5):S902-S906. doi: 10.4103/IJO.IJO_2560_23. Epub 2024 May 20.
PURPOSE
To explore the clinicopathological characteristics of immunoglobulin G4 (IgG4)-positive ocular adnexal marginal zone B-cell lymphoma (OAML) and associated patient treatment outcomes.
METHODS
Medical records from patients diagnosed with IgG4-positive OAML treated at the West China Hospital between January 2016 and August 2023 were retrospectively analyzed.
RESULTS
This study included data from 22 patients (11 males, 11 females), aged between 36 and 83 years, with disease durations from 1 month to 30 years. Sixteen cases exhibited unilateral ocular involvement (ten left eyes, six right eyes), while six exhibited bilateral involvement. Common clinical symptoms included ocular masses, eyelid swelling, and proptosis, with the orbit and lacrimal gland being the most commonly impacted sites. Among the 22 patients, 13 who were clinically suspected of having IgG4-related ophthalmic disease (IgG4-ROD) underwent serum IgG4 testing pre-operatively, revealing elevated IgG4 levels in 11 of these patients. The use of computed tomography and magnetic resonance imaging facilitated the evaluation of the location and size of lesions. All 22 patients received surgical treatment. Subsequently, 14 of these patients underwent local radiotherapy, five received post-operative chemotherapy, and three were closely observed. The follow-up period of patients in this study was 3-77 months, with an average follow-up time of 36 months. Except for one patient who died of disease progression, all others showed favorable prognoses with significant improvements.
CONCLUSIONS
These results support the classification of IgG4-positive OAML as a distinct OAML sub-type with clinical features that partially overlap with IgG4-ROD. Therefore, accurate differentiation between OAML and IgG4-ROD is imperative, necessitating timely surgical intervention and precise pathological diagnosis to prevent diagnostic errors and inappropriate treatment. Currently, no standardized treatments for IgG4-positive OAML exist, but our results suggest that standard OAML therapies are generally efficacious.
目的
探讨 IgG4 阳性眼附属器边缘区 B 细胞淋巴瘤（OAML）的临床病理特征及其与患者治疗结局的关系。
方法
回顾性分析 2016 年 1 月至 2023 年 8 月在华西医院接受治疗的 IgG4 阳性 OAML 患者的病历资料。
结果
本研究共纳入 22 例患者（男 11 例，女 11 例），年龄 3683 岁，病程 1 个月至 30 年。16 例为单侧眼部受累（左眼 10 例，右眼 6 例），6 例为双侧受累。常见临床表现包括眼部肿块、眼睑肿胀和眼球突出，最常累及的部位是眼眶和泪腺。22 例患者中，13 例临床疑诊 IgG4 相关眼病（IgG4-ROD）患者术前进行了血清 IgG4 检测，其中 11 例 IgG4 升高。计算机断层扫描和磁共振成像有助于评估病变的位置和大小。所有 22 例患者均接受了手术治疗。随后，14 例患者接受局部放疗，5 例患者接受术后化疗，3 例患者密切观察。本研究患者的随访时间为 377 个月，平均随访时间为 36 个月。除 1 例患者因疾病进展死亡外，其余患者预后良好，症状均显著改善。
结论
这些结果支持将 IgG4 阳性 OAML 归类为一种独特的 OAML 亚型，其临床特征部分与 IgG4-ROD 重叠。因此，准确区分 OAML 和 IgG4-ROD 至关重要，需要及时进行手术干预和精确的病理诊断，以避免误诊和不恰当的治疗。目前，尚无针对 IgG4 阳性 OAML 的标准化治疗方法，但我们的结果表明，标准的 OAML 治疗方法通常有效。
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