十二指肠型滤泡性淋巴瘤临床病理学特征

Clinicopathological features of duodenal-type follicular lymphoma

目的 探讨十二指肠型滤泡性淋巴瘤(DFL)的临床病理学特征。方法 收集新疆维吾尔自治区人民医院2019-10—2020-11确诊的3例DFL患者的临床症状、内镜表现、实验室检查、影像学检查等临床资料,分析其镜下形态、免疫组化表型特征、治疗及随访情况。结果 3例患者中2例女性,1例男性,年龄47~79岁。病变均位于十二指肠,内镜下3例均显示黏膜粗糙,黏膜结构不清,其中1例表面呈结节样改变。1例常规做内镜体检时无意中发现,其余2例有腹部不适,腹痛、腹胀、纳差等症状。镜下黏膜固有层及黏膜下层均见核形略不规则的中心细胞样细胞浸润,多个界限清晰的异常淋巴滤泡,形态学均符合1级病变。免疫组化显示Bcl-2异常表达于肿瘤性滤泡,CD21和CD23标记FDC呈“十二指肠模式”,表现为滤泡中心边缘更加浓聚及染色更深。两例患者选择“等待和观察”,未做特殊治疗,例3患者行利妥昔单抗联合化疗,3例患者均病情平稳,未见进展。结论 DFL是FL的独特亚型,形态学上类似于低级别FL,肿瘤性滤泡表达Bcl-2、CD21和CD23标记FDC呈特征性的“十二指肠模式”。大多数DFL患者即使未经治疗也具有良好的预后,但是建议进行长期随访,因为少数病例有发生组织学转变为高级别淋巴瘤的风险。

Objective To investigate the clinicopathological features of duodenal-type follicular lymphoma.Methods Three cases of duodenal-type follicular lymphoma diagnosed at People’s Hospital of Xinjiang Uygur Autonomous Region from 2019 to 2020 with detailed clinical data,including clinical symptoms,endoscopic performance,laboratory examinations and imageological diagnosis.The histomorphology,immunophenotype,treatment and prognoses were analyzed.Results Two of the three patients were females and one was male,aged 47-79 years.Among the 3 patients,the lesions were all located in the duodenum.Under endoscope,all the 3 patients showed rough mucosa with unclear mucosal structure,and 1 of them showed nodular changes on the surface.One case was unintentionally found during routine endoscopy,while the other two cases had abdominal discomfort,abdominal pain,abdominal distension,and other symptoms.Microscopically,both lamina propria and submucosa showed centrocyte-like infiltration with slightly irregular nuclei and multiple abnormal lymphatic follicles with clear boundaries,constituting grade 1 disease based on nodal follicular lymphoma.Immunohistochemistry showed that Bcl-2 was abnormally expressed in neoplastic follicles,and CD21 and CD23 labeled FDCs presented a “duodenal pattern” with more concentrated and more stained margins at the center of the follicles.Two patients chose “wait and watch” and did not receive special treatment.Case 3 received rituximab combined with chemotherapy,and the condition of the three patients was stable without any progress.Conclusion DFL is a distinct subtype of FL,morphologically similar to low-grade FL;tumor follicles express Bcl-2,and CD21 and CD23 labeled FDCs,showing a characteristic “duodenal pattern”.Most patients with DFL have a good prognosis even if they are not treated,but long-term follow-up is recommended because a small number of cases are at risk of histologic progression to high-grade lymphoma.