American journal of ophthalmology · 2026
IgG4-Positive Ocular Adnexal Extranodal Marginal Zone Lymphoma as an Aggressive Clinical Subtype: Evidence From a 377-Patient Cohort.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Sun Hetian, Tang Junjie, Li Jinmiao, Gao Yang, Huang Weifeng, Zhang Zhihui, Wang Meng, Liu Yaoming, Su Shicai, Yu Bing, Chen Shuxia, Tang Lijuan, Zhang Ping, Ai Siming, Mao Yuxiang, Lu Rong
Аннотация
OBJECTIVE: To investigate the clinicopathological characteristics of IgG4-positive ocular adnexal lymphoma (OAL).
DESIGN: Retrospective clinical cohort study.
PARTICIPANTS: A total of 377 patients diagnosed with OAL between October 2014 and October 2024 were enrolled. Study data were analyzed from March 2025 to August 2025.
METHODS: A comprehensive review of the medical records for all OAL patients was conducted. The distribution of IgG4-positive cases was assessed. Clinicopathological characteristics, as well as recurrence risk, were compared between IgG4-positive and IgG4-negative patients.
MAIN OUTCOMES AND MEASURES: Differences in local and systemic involvement at baseline, and recurrence risk during follow-up.
RESULTS: The highest proportion of IgG4-positive patients was observed in extranodal marginal zone lymphoma (EMZL) (23.3% [75/322]), with a significant higher frequency of involvement in the eyelid, lacrimal gland, optic nerve, superior/inferior orbital fissures, cavernous sinus, and Infratemporal fossa/pterygopalatine fossa compared with IgG4-negative EMZL patients. At presentation, T3-T4 stages were more frequently observed among IgG4-positive EMZL patients, while differences in systemic involvement showed no statistical significance after Benjamini-Hochberg adjustment. The Ki-67 index was higher in IgG4-positive EMZL patients (20.08 ± 6.56 vs 14.13 ± 5.03, P < .0001), and IgG4-positive plasma cells showed significant positive correlations with T-stage and Ki-67 index. Both IgG4-positive and IgG4-negative EMZL patients were primarily treated with radiotherapy following surgery. IgG4-positive EMZL patients experienced earlier recurrence (2.04 ± 1.21 years vs 3.71 ± 1.23 years, P = .002) and a lower cumulative proportion without recurrence or reoperation (All P < .01). Cox regression analysis revealed a strong association between IgG4-positive EMZL and recurrence in multivariate model (HR, 2.04, 95% CI, 1.07-3.91, P = .031). IgG4-positive plasma cells in tissue were significantly negatively correlated with the time to recurrence (rs = -0.605, P < .0001).
CONCLUSIONS: IgG4-positive EMZL represents a more aggressive clinical subtype, characterized by more widespread local involvement and an increased risk of recurrence, thereby warranting more intensive management and long-term follow-up for disease surveillance. Targeted management strategies need to be established to achieve optimal disease control.
DESIGN: Retrospective clinical cohort study.
PARTICIPANTS: A total of 377 patients diagnosed with OAL between October 2014 and October 2024 were enrolled. Study data were analyzed from March 2025 to August 2025.
METHODS: A comprehensive review of the medical records for all OAL patients was conducted. The distribution of IgG4-positive cases was assessed. Clinicopathological characteristics, as well as recurrence risk, were compared between IgG4-positive and IgG4-negative patients.
MAIN OUTCOMES AND MEASURES: Differences in local and systemic involvement at baseline, and recurrence risk during follow-up.
RESULTS: The highest proportion of IgG4-positive patients was observed in extranodal marginal zone lymphoma (EMZL) (23.3% [75/322]), with a significant higher frequency of involvement in the eyelid, lacrimal gland, optic nerve, superior/inferior orbital fissures, cavernous sinus, and Infratemporal fossa/pterygopalatine fossa compared with IgG4-negative EMZL patients. At presentation, T3-T4 stages were more frequently observed among IgG4-positive EMZL patients, while differences in systemic involvement showed no statistical significance after Benjamini-Hochberg adjustment. The Ki-67 index was higher in IgG4-positive EMZL patients (20.08 ± 6.56 vs 14.13 ± 5.03, P < .0001), and IgG4-positive plasma cells showed significant positive correlations with T-stage and Ki-67 index. Both IgG4-positive and IgG4-negative EMZL patients were primarily treated with radiotherapy following surgery. IgG4-positive EMZL patients experienced earlier recurrence (2.04 ± 1.21 years vs 3.71 ± 1.23 years, P = .002) and a lower cumulative proportion without recurrence or reoperation (All P < .01). Cox regression analysis revealed a strong association between IgG4-positive EMZL and recurrence in multivariate model (HR, 2.04, 95% CI, 1.07-3.91, P = .031). IgG4-positive plasma cells in tissue were significantly negatively correlated with the time to recurrence (rs = -0.605, P < .0001).
CONCLUSIONS: IgG4-positive EMZL represents a more aggressive clinical subtype, characterized by more widespread local involvement and an increased risk of recurrence, thereby warranting more intensive management and long-term follow-up for disease surveillance. Targeted management strategies need to be established to achieve optimal disease control.