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摘要: 总结4例十二指肠型滤泡性淋巴瘤(duodenal type follicular lymphoma，DFL)患者的临床表现、病理学特征、治疗及预后。回顾性分析青岛大学附属医院2018年1月至2022年2月确诊并治疗的4例DFL患者的临床资料，并进行相关文献复习。4例患者中男3例，女1例；中位年龄52.5(38~70)岁。4例患者均行内镜检查，病变部位均位于十二指肠降段。1例患者合并幽门螺杆菌感染。治疗方面，2例患者随访观察，1例患者以R-COP方案作为一线化疗方案，应用4个疗程后改为利妥昔单抗单药治疗，1例患者采用奥妥珠单抗治疗。1例患者复查胃镜显示十二指肠降部仍有病变，2例患者疾病未进展，1例患者疾病完全缓解。DFL是一种惰性的、进展缓慢的淋巴瘤，大多数DFL是在检查中偶然发现，多数患者临床症状不明显，部分患者可能出现腹部不适。该疾病总体预后较好，无进展生存期和总生存期高。目前尚无关于DFL最佳治疗方案的明确建议，治疗选择包括随访观察、利妥昔单抗单药治疗以及联合化疗和放疗等。
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关键词:
- 十二指肠型滤泡性淋巴瘤 /
- 临床表现 /
- 病理学特征 /
- 治疗 /
- 预后
Abstract: To summarize 4 cases of duodenal type follicular lymphoma(DFL) and to explore its clinical features, pathological characteristics, treatment, and prognosis, the clinical data of four patients with DFL were collected and analyzed from the Affiliated Hospital of Qingdao University during January 2018 to February 2022. The clinical features, histopathological and immunohistochemical characteristics, treatment and prognosis were retrospectively analyzed, with review of related literatures. Four patients included 3 males and 1 female, with a median age of 52.5 years(range 38-70). All patients underwent the endoscopic examination, and the lesions were located at the descending part of duodenum. One patient was infected with Helicobacter pylori. In terms of treatment, 2 patients took a "watch and wait" strategy, 1 patient was treated with rituximab monotherapy after 4 courses of R-COP chemotherapy, and 1 patient treated with obinutuzumab. Review of gastroscopy showed that 1 patient still had lesions in the descending duodenum, 2 patients had no disease progression, and 1 patient reached complete response. DFL is an indolent, slow-progressing lymphoma, which is found mainly by chance during examinations. Most patients have insignificant clinical symptoms, and some cases may present with abdominal discomfort. The overall prognosis of the disease is good, with high progression-free survival and overall survival. At present, there is no clear recommendation on the optimal treatment regimen for DFL. Treatment options include "watch and wait" strategy, rituximab monotherapy, combined chemotherapy, and radiation therapy. -
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表 1 4例DFL患者的临床资料及治疗随访情况
病例 性别 年龄/岁 临床表现 病变部位 内镜所见 幽门螺杆菌 治疗(药物×疗程) 随访 1 女 63 腹胀半年，盗汗1个月 十二指肠降段 十二指肠降部乳头附近见多发黏膜白斑 + 随访观察+抗幽门螺杆菌治疗 复查胃镜显示十二指肠降部黏膜处白色点状隆起较前增多 2 男 38 上腹部不适1年，夜间偶有盗汗，体重下降明显 十二指肠降段 十二指肠降段乳头远端内侧壁见一处黏膜粗糙，局部略凹陷糜烂 未检测 随访观察 疾病未进展 3 男 70 胃部隐痛、腹胀1个月余 十二指肠降段 十二指肠降段内侧壁见片状黏膜粗糙区域 - R-COP方案×4 +利妥昔单抗×4 疾病未进展 4 男 42 偶有腹痛 十二指肠降段 十二指肠降段乳头周围6点至12点方向可见宽度约2 cm黏膜不规则隆起，呈铺路石样改变 - 奥妥珠单抗×5 疾病完全缓解 
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表 2 4例DFL患者的免疫表型
病例 CD10 CD20 CD21 CD23 CD3 CD5 CD31 Bcl-2 Bcl-6 Ki-67 Pax-5 Cyclin D1 1 + + + 无资料 - 无资料 无资料 + 弱+ 约20% 无资料 无资料 2 + + + 无资料 T细胞+ - 血管+ + 滤泡中心+ 热点区+ 5% + - 3 + 弥漫+ + 无资料 - - 无资料 + + 20% 无资料 - 4 + 弥漫+ + + 少量+ 无资料 无资料 + + 肿瘤性滤泡30%，滤泡间5% 无资料 无资料
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