25例原发性肠道T细胞淋巴瘤的临床、病理及内镜特点分析
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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.
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.
引文
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[13] Kim JH,Lee JH,Lee J,et al.Primary NK-/T-cell lymphoma of the gastrointestinal tract:clinical characteristics and endoscopic findings[J].Endoscopy,2007,39:156-160.
[14] Torosian MH,Turnbull AD.Emergency laparotomy for spontaneous intestinal and colonic perforations in cancer patients receiving corticosteroids and chemotherapy[J].J Clin Oncol,1988,6:291-296.
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[2] Chen Y,Chen Y,Chen S,et al.Primary gastrointestinal lymphoma:a retrospective multicenter clinical study of 415 cases in Chinese province of Guangdong and a systematic review containing 5075 Chinese patients[J].Medicine (Baltimore),2015,94:e2119.
[3] Nakamura S,Matsumoto T.Gastrointestinal lymphoma:recent advances in diagnosis and treatment[J].Digestion,2013,87:182-188.
[4] Swerdlow SH,Campo E,Pileri SA,et al.The 2016 revision of the World Health Organization classification of lymphoid neoplasms[J].Blood,2016,127:2375-2390.
[5] Kim DH,Lee D,Kim JW,et al.Endoscopic and clinical analysis of primary T cell lymphoma of the gastrointestinal tract according to pathological subtype[J].J Gastroenterol Hepatol,2014,29:934-943.
[6] Kim SJ,Choi CW,Mun YC,et al.Multicenter retrospective analysis of 581 patients with primary intestinal non-hodgkin lymphoma from the Consortium for Improving Survival of Lymphoma (CISL)[J].BMC Cancer,2011,11:321.
[7] Tse E,Gill H,Loong F,et al.Type Ⅱ enteropathy-associated T-cell lymphoma:a multicenter analysis from the Asia Lymphoma Study Group[J].Am J Hematol,2012,87:663-668.
[8] Jiang M,Chen X,Yi Z,et al.Prognostic characteristics of gastrointestinal tract NK/T-cell lymphoma:an analysis of 47 patients in China[J].J Clin Gastroenterol,2013,47:e74-79.
[9] 王亚楠,李骥,倪岳晖,等.内镜下轻微病变的单形性嗜上皮性肠道T细胞淋巴瘤的临床特点[J].中华内科杂志,2018,57:112-117.
[10] Tang XF,Yang L,Duan S,et al.Intestinal T-cell and NK/T-cell lymphomas:a clinicopathological study of 27 Chinese patients[J].Ann Diagn Pathol,2018,37:107-117.
[11] Ishibashi H,Nimura S,Kayashima Y,et al.Multiple lesions of gastrointestinal tract invasion by monomorphic epitheliotropic intestinal T cell lymphoma,accompanied by duodenal and intestinal enteropathy like lesions and microscopic lymphocytic proctocolitis:a case series[J].Diagn Pathol,2016,11:66.
[12] Fukushima M,Kawanami C,Inoue S,et al.Enteropathy-associated T-cell lymphoma diagnosed and followed-up by using double-balloon enteroscopy[J].Gastrointest Endosc,2013,78:361-362.
[13] Kim JH,Lee JH,Lee J,et al.Primary NK-/T-cell lymphoma of the gastrointestinal tract:clinical characteristics and endoscopic findings[J].Endoscopy,2007,39:156-160.
[14] Torosian MH,Turnbull AD.Emergency laparotomy for spontaneous intestinal and colonic perforations in cancer patients receiving corticosteroids and chemotherapy[J].J Clin Oncol,1988,6:291-296.
[15] Vaidya R,Habermann TM,Donohue JH,et al.Bowel perforation in intestinal lymphoma:incidence and clinical features[J].Ann Oncol,2013,24:2439-2443.
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