Shao S H, Gu H Y, Lin D L, Shi H L, Zhang Y J, Li Y J
Department of Pathology, the Affiliated Hospital of Qingdao University, Qingdao 266555, China.
Zhonghua Bing Li Xue Za Zhi. 2019 Oct 8;48(10):762-766. doi: 10.3760/cma.j.issn.0529-5807.2019.10.003.
To investigate the clinicopathological features of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract. Five cases of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract from the Affiliated Hospital of Qingdao University from 2016 to 2019 were retrospectively reviewed. The clinical and pathological parameters were analyzed by combining clinical data and reviewing the available literature of 35 cases (34 cases abroad and 1 case in China). There were 4 males and 1 female with a median age of 47 years (18-66 years). All patients had abdominal pain and constitutional symptoms including diarrhea, emaciation, intermittent mucous stool or oral and epiglottic ulcers. Endoscopic manifestations included multiple punctate congestion, erosion and ulcer at the terminal ileum and colorectum. Two cases had congestion and erosion of antrum and angle of stomach, and the lesions did not fuse and form tumors. Histologically, the lamina propria was expanded by a dense, medium to small lymphocyte infiltration, which was monomorphic, with slightly irregular nuclei without prominent nucleolus or lymphoepithelial lesions. There were admixed small amount of plasma cells and eosinophils. In 4 cases, immunohistochemistry showed the lesional cells were positive for CD3, CD8, TIA1, and negative for CD4, CD56, granzyme B and Ki-67 index was ≤10%. In situ hybridization showed that EBER was negative and clonal TCR gene rearrangement was detected. One consultation case was CD3(+), CD5(-) and Ki-67 index of 10%, although other indicators were not done. All five patients were treated with symptomatic support. In follow-up observation for 2 to 25 months, all patients were alive with the disease. Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract is a newly classified monoclonal T-cell proliferative disease, with low incidence, clinical inertia and long-term survival. It has unique clinicopathological features but pathologically it is easily misdiagnosed as inflammatory bowel disease or T-cell lymphoma. Correct diagnosis is of great important clinical significance.
探讨胃肠道惰性T细胞淋巴增殖性疾病的临床病理特征。回顾性分析2016年至2019年青岛大学附属医院收治的5例胃肠道惰性T细胞淋巴增殖性疾病患者的临床资料，并结合35例（国外34例，国内1例）相关文献复习分析其临床及病理参数。患者中男性4例，女性1例，中位年龄47岁（18 - 66岁）。所有患者均有腹痛及全身症状，包括腹泻、消瘦、间断黏液便或口腔及会厌溃疡。内镜表现为回肠末端及结直肠多发点状充血、糜烂及溃疡。2例患者胃窦及胃角有充血糜烂，病变未融合成瘤。组织学表现为固有层被密集的中小淋巴细胞浸润所扩张，细胞呈单形性，核略不规则，无明显核仁及淋巴上皮病变，混有少量浆细胞及嗜酸性粒细胞。4例患者免疫组化显示病变细胞CD3、CD8、TIA1阳性，CD4、CD56、颗粒酶B阴性，Ki-67指数≤10%。原位杂交显示EBER阴性，检测到克隆性TCR基因重排。1例会诊病例CD3(+)、CD5(-)，Ki-67指数为10%，其他指标未检测。5例患者均给予对症支持治疗。随访观察2至25个月，所有患者均存活。胃肠道惰性T细胞淋巴增殖性疾病是一种新分类的单克隆T细胞增殖性疾病，发病率低，临床进程惰性，生存期长。具有独特的临床病理特征，但病理上易误诊为炎症性肠病或T细胞淋巴瘤。正确诊断具有重要的临床意义。
