Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, 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, China.
State Key Laboratory of Experimental Hematology and Division of Pediatric Blood Diseases Center, Institute of Hematology and Blood Diseases Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Tianjin, China.
Br J Haematol. 2024 May;204(5):1771-1779. doi: 10.1111/bjh.19393. Epub 2024 Mar 6.
Primary gastrointestinal follicular lymphoma (PGI-FL) is a rare extra-nodal lymphoma. Its epidemiology and prognosis remain unclear. We performed a retrospective analysis of eligible patients with 1648 PGI-FL and 34 892 nodal FL (N-FL) in the Surveillance, Epidemiology and End Results (SEER) database. The age-adjusted average annual incidence of PGI-FL was 0.111/100000. The median overall survival (OS) for PGI-FL and N-FL patients was 207 and 165 months respectively. The 5-year diffuse large B-cell lymphoma (DLBCL) transformation rates were 2.1% and 2.6% respectively. Age, sex, grade, Ann Arbor stage, primary site and radiation were independent prognostic factors (p < 0.05). Nomograms were constructed to predict 1-, 5- and 10-year OS and disease-specific survival (DSS). The receiver operating characteristic curves and calibration plots showed the established nomograms had robust and accurate performance. Patients were classified into three risk groups according to nomogram score. In conclusion, the incidence of PGI-FL has increased over the past 40 years, and PGI-FL has a better prognosis and a lower DLBCL transformation rate than N-FL. The nomograms were developed and validated as an individualized tool to predict survival. Patients were divided into three risk groups to assist clinicians in identifying high-risk patients and choosing the optimal individualized treatments.
原发性胃肠道滤泡性淋巴瘤（PGI-FL）是一种罕见的结外淋巴瘤。其流行病学和预后尚不清楚。我们对 SEER 数据库中符合条件的 1648 例 PGI-FL 和 34892 例结内滤泡性淋巴瘤（N-FL）患者进行了回顾性分析。年龄调整后的 PGI-FL 平均年发病率为 0.111/100000。PGI-FL 和 N-FL 患者的中位总生存期（OS）分别为 207 和 165 个月。5 年弥漫性大 B 细胞淋巴瘤（DLBCL）转化率分别为 2.1%和 2.6%。年龄、性别、分级、Ann Arbor 分期、原发部位和放疗是独立的预后因素（p<0.05）。构建了列线图来预测 1、5 和 10 年 OS 和疾病特异性生存（DSS）。ROC 曲线和校准图显示建立的列线图具有稳健和准确的性能。根据列线图评分将患者分为三个风险组。总之，过去 40 年来 PGI-FL 的发病率有所增加，PGI-FL 的预后优于 N-FL，DLBCL 转化率较低。列线图是作为一种个体化工具来预测生存而开发和验证的。将患者分为三个风险组，以帮助临床医生识别高危患者并选择最佳个体化治疗。
