摘要目的 探讨十二指肠型滤泡性淋巴瘤的临床病理特征及治疗和预后情况.方法 收集广东省人民医院2014至2015年诊断的4例十二指肠型滤泡性淋巴瘤患者的临床信息,分析其组织学形态、免疫表型特征及治疗和预后情况.结果 患者年龄51～57岁,平均年龄54岁;男女各2例;病变部位分别为胃底1例、十二指肠降段2例、回肠末端1例,内镜下表现为多发丘状或颗粒状隆起.组织学形态:黏膜层见多个淋巴滤泡增生,相当于低级别滤泡性淋巴瘤的形态.免疫组织化学结果:肿瘤细胞表达CD20、CD10、bcl-6及bcl-2,CD21显示滤泡树突细胞网架位于滤泡外围,呈环状包绕滤泡;Ki-67阳性指数较低.3例患者经利妥昔单抗治疗4个疗程后病变完全消退.1例患者未治疗,在诊断15个月后进展为系统性淋巴瘤.结论 十二指肠型滤泡性淋巴瘤属于低度恶性的特殊类型的滤泡性淋巴瘤;组织学表现为低级别滤泡性淋巴瘤形态,滤泡树突网架特征性地位于滤泡外围,阳性指数低;绝大部分预后良好,利妥昔单抗治疗疗效好,部分患者有进展为系统性淋巴瘤风险.

abstractsObjective To investigate the clinicopathological features,treatment and prognosis of duodenal-type follicular lymphoma.Methods Four cases of duodenal-type follicular lymphoma diagnosed at Guangdong General Hospital from 2014 to 2015 with detailed clinical data were included.The histomorphology,immunophenotype,treatment and prognoses were analyzed.Results The patients' age ranged from 51 to 57 years (mean 54 years),and there were 2 males and 2 females.The involved sites were gastric fundus in one case,second portion of the duodenum in two cases and terminal ileum in one case.All patients presented with multiple mucosal granules or nodules at endoscopy.Microscopically,there were multiple mucosal neoplastic follicles,constituting grade 1-2 disease based on nodal follicular lymphoma grading system.The tumor cells were positive for CD20,CD10,bcl-6 and bcl-2.CD21 highlighted the follicular dendritic meshwork mainly at the periphery of the follicles.Proliferation index was low.Three patients received rituximab monotherapy for 4 cycles,leading to complete remission.One patient refused therapy and the disease progressed to systemic lymphoma 15 months after the initial diagnosis.Conclusions Duodenal-type follicular lymphoma is a special variant of follicular lymphoma with indolent clinical course.The tumor exhibits morphology of low grade follicular lymphoma with characteristic dendritic meshwork at the periphery of the follicles and a low proliferation index.Prognosis is excellent.Rituximab monotherapy is treatment of choice,but a small minority of patients may progress to systemic disease.