摘要
目的通过分析原发性肠道T细胞淋巴瘤(PITL)患者的临床、病理和内镜特点,提高对该类疾病的认识和诊断水平。方法筛选2012年8月至2018年8月于北京协和医院住院并经病理确诊为PITL的患者25例,采集其临床信息,进行回顾性分析。结果25例PITL中,单形性嗜上皮性肠道T细胞淋巴瘤(MEITL)9例,结外NK/T淋巴瘤(ENKTL)5例,肠道T细胞淋巴瘤-非特指型(ITL-NOS)5例,外周T细胞性淋巴瘤(PTL)4例,间变性大细胞淋巴瘤ALK阳性和T淋巴母细胞淋巴瘤各1例;男女比例为4∶1,中位年龄为45(13~82)岁,中位病程为5(0.25~108)个月;常见症状为体质量明显下降(72%)、腹痛(64%)、腹泻(64%)、发热(64%);22例患者行内镜检查,病灶呈溃疡型13例(59.1%)、侵袭型7例(31.8%)、隆起型2例(9.1%),内镜活检的确诊率为54.5%(12/22),首次活检确诊率仅为31.8%(7/22);25例患者共行17人次手术,其中11次为肠穿孔或消化道大出血后的急诊手术,急腹症发生率为44%(11/25);MEITL患者在发病年龄、体质量下降、LDH水平、EBV感染、hsCRP水平、内镜活检确诊率、病变累及范围及病变内镜表现方面与其他PITL患者存在差异,有统计学意义。结论PITL的常见内镜表现为溃疡型和侵袭型,单次活检确诊率低,对疑诊患者应多次多部位活检以提高诊断率;MEITL是PITL中最常见的病理类型,其临床及内镜特点不同于其他PITL。
Objective To explore the clinical, pathological and endoscopic features of primary intestinal T-cell lymphomas(PITL), and to raise understanding and diagnostic levels of this disease. Methods Clinical data of 25 hospitalized patients diagnosed with PITL in Peking Union Medical College Hospital from Aug. 2012 to Aug. 2018 were retrospectively analyzed. Results In the 25 patients, 9 with monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL), 5 with extranodal NK-/T-cell lymphoma(ENKTL), 5 with intestinal T-cell lymphoma-not otherwise specified(ITL-NOS), 4 with peripheral T-cell lymphoma(PTL), 1 with anaplastic large cell lymphomas and 1 with T-cell lymphoblastic lymphoma. The male-to-female ratio was 4∶1, with the median age of 45 years(range,13 to 82 years) and the median disease duration of 5 months(range,0.25 to 108 months). The common clinical manifestations included weight loss(72%), abdominal pain(64%), diarrhea(64%) and fever(64%). Endoscopic features of 22 patients included 13(59.1%) with ulcerative features, 7(31.8%) with infiltrative features, and 2(9.1%) with protrusive features. Twelve out of 22 patients(54.5%) were diagnosed by endoscopic biopsies, and only 7(31.8%) were diagnosed at the first biopsy. Altogether 17 operations were performed, including 11 emergent operations due to complications(perforation and/or bleeding). Significant differences were found between MEITL and other subtypes in age of onset, weight loss, LDH level, EBV infection, hsCRP, site of involvement, diagnostic rate by endoscopic biopsy and endoscopic features. Conclusions Ulcerative and infiltrative features are common endoscopic findings of PITL. Since diagnostic rate by the first biopsy is low, more biopsies are needed in the suspected patients to make diagnosis. The clinical characteristics of MEITL are different from other subtypes.
作者
舒慧君
杨红
周炜洵
李骥
费贵军
王强
钱家鸣
SHU Hui-jun;YANG Hong;ZHOU Wei-xun;LI Ji;FEI Gui-jun;WANG Qiang;QIAN Jia-ming(Department of Gastroenterology,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China;Department of Pathology,Peking Union Medical College Hospital,Peking Union Medical College,Chinese Academy of Medical Sciences,Beijing 100730,China)
出处
《基础医学与临床》
CSCD
2019年第6期871-876,共6页
Basic and Clinical Medicine
基金
北京协和医学院本科教育教学改革立项项目(2018zlgc0113)
关键词
原发性肠道T细胞淋巴瘤
病理类型
内镜特点
临床特点
primary intestinal T-cell lymphoma
pathological subtype
endoscopic feature
clinical characteristics
作者简介
通信作者:钱家鸣:qianjiaming1957@126.com.
