Wang Ganggang, Wang Jiesong, Sun Cong, Yu Jingwei, Lv Chao, Song Zheng, Han Xue, Li Lanfang, Qiu Lihua, Qian Zhengzi, Zhou Shiyong, Liu Xia, Wang Xianhuo, He Jin, Zhang Huilai
State Key Laboratory of Druggability Evaluation and Systematic Translational Medicine/Department of Lymphoma, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, the Sino-US Center for Lymphoma and Leukemia Research, Tianjin, CN.
Cancer Center, Shanxi Bethune Hospital, Shanxi Academy of Medical Science, Tongji Shanxi Hospital, Third Hospital of Shanxi Medical University, Taiyuan, China.
Hematol Oncol. 2025 Jul;43(4):e70115. doi: 10.1002/hon.70115.
Primary large B-cell lymphoma of immune-privileged sites (IP-LBCL), a recently defined entity in WHO-HAEM5, includes primary diffuse large B-cell lymphoma (DLBCL) occurring in immune-privileged areas like the central nervous system (PCNS-LBCL), vitreoretinal system (PVR-LBCL), and testis (PT-LBCL) in immunocompetent patients. This study aimed to identify prognostic factors and create a predictive model for IP-LBCL. We analyzed 213 newly diagnosed IP-LBCL patients from April 2006 to April 2023. A nomogram and prognostic index, IPLBCL-PI, were developed based on elevated LDH, ECOG ≥ 2, and PCNS-LBCL subtype as independent risk factors for poorer PFS. IPLBCL-PI categorized patients into four risk groups: low, low-intermediate, intermediate-high, and high. The model effectively predicted both PFS and OS in the training cohort and was validated in two external centers. Subgroup analyses showed that IPLBCL-PI outperformed the Nottingham/Barcelona (NB) and Memorial Sloan Kettering Cancer Center (MSKCC) models in PCNS-LBCL and was comparable to the International Prognostic Index (IPI) in PT-LBCL. IPLBCL-PI is the first prognostic model for IP-LBCL, offering risk stratification and aiding clinical decision-making for this rare entity.
免疫豁免部位原发性大B细胞淋巴瘤（IP-LBCL）是世界卫生组织血液肿瘤分类第5版（WHO-HAEM5）中最近定义的一种疾病实体，包括免疫功能正常患者发生于免疫豁免区域（如中枢神经系统（PCNS-LBCL）、玻璃体视网膜系统（PVR-LBCL）和睾丸（PT-LBCL））的原发性弥漫性大B细胞淋巴瘤（DLBCL）。本研究旨在确定IP-LBCL的预后因素并建立预测模型。我们分析了2006年4月至2023年4月期间213例新诊断的IP-LBCL患者。基于乳酸脱氢酶升高、东部肿瘤协作组（ECOG）评分≥2以及PCNS-LBCL亚型作为无进展生存期较差的独立危险因素，开发了一种列线图和预后指数IPLBCL-PI。IPLBCL-PI将患者分为四个风险组：低、低中、高中和高。该模型在训练队列中有效地预测了无进展生存期（PFS）和总生存期（OS），并在两个外部中心得到验证。亚组分析显示，在PCNS-LBCL中，IPLBCL-PI的表现优于诺丁汉/巴塞罗那（NB）模型和纪念斯隆凯特琳癌症中心（MSKCC）模型，在PT-LBCL中与国际预后指数（IPI）相当。IPLBCL-PI是首个针对IP-LBCL的预后模型，可为这一罕见疾病实体提供风险分层并辅助临床决策。
