18例十二指肠型滤泡性淋巴瘤的临床病理学研究

Studies on clinicopathological features of duodenal-type follicular lymphoma of 18 patients

为研究十二指肠型滤泡性淋巴瘤(duodenal-type follicular lymphoma,D-FL)的临床及病理学特点,总结2020年1月至2023年7月期间首都医科大学附属北京天坛医院病理科会诊及本院确诊的18例D-FL患者的临床症状、内镜特点、病理形态学特点、免疫表型、分子病理特征以及治疗随访经过。18例D-FL患者,男10例,女8例,中位年龄为49(32~69)岁。患者多为胃肠镜检查发现或出现常见胃肠道症状,如胃痛、反酸、呕吐及腹泻。内镜大多表现为多发灰白色小息肉样隆起。病理形态学上为黏膜层及黏膜下层可见淋巴滤泡样结构。免疫表型为肿瘤细胞广泛强表达CD20及BCL2,增殖活性低。免疫球蛋白克隆分析显示1例IgK单克隆重排(1/1),FISH检测1例BCL2基因重排(1/3)。所有患者均未进行靶向及化疗,采取等待及观望策略。中位随访12(2~34)个月。研究显示D-FL属于惰性淋巴瘤,好发于十二指肠,预后良好。

To investigate the clinical and pathological characteristics of duodenal-type follicular lymphoma(D-FL),and to deepen the understanding of Duodenal-type follicular lymphoma.The clinical symptoms,endoscopic features,pathologic features,immunophenotype,molecular pathological features and treatment follow-up of 18 D-FL patients diagnosed in Department of Pathology,Beijing Tiantan Hospital affiliated to Capital Medical University between January 2020 and July 2023 were summarized.A total of 18 patients with D-FL were included,including 10 males and 8 females.The median age was 49(32-69)years respectively.Most of the patients were found during gastroenteroscopy or presented with the common gastrointestinal symptoms of stomach pain,acid reflux,vomiting and diarrhea.Most endoscopic findings were multiple small gray and white polyposis.In the pathological morphology,the mucous layer and submucous layer showed lymphoid follicular structures with full and strained follicles.The immunophenotype showed that the tumor cells strongly expressed CD20 and BCL2 and had low proliferation activity.Immunoglobulin clonal analysis of 1 case showed IgK monoclonal rearrangement(1/1).FISH showed 1 case of BCL2 gene rearrangement(1/3).All patients did not receive targeted chemotherapy and adopted a wait-and-see strategy.Median follow-up was 12(2-34)months.This study shows that D-FL is an indolent lymphoma,which tends to occur in the duodenum and has a good prognosis.