Department of Gastroenterology, Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou 310000, Zhejiang Province, China.
World J Gastroenterol. 2020 Jul 21;26(27):3989-3997. doi: 10.3748/wjg.v26.i27.3989.
The incidence of intestinal NK/T cell lymphoma (NKTCL) is extremely low, and the clinical symptoms are atypical, which makes it difficult to distinguish this disorder from Crohn's disease (CD), T lymphocyte proliferative disease, and other immune disorders. The misdiagnosis rate is high, and the patient's prognosis is poor.
In this case, the patient had repeated high fever, colonoscopy revealed multiple ulcers, and the initial diagnosis was CD. The patient's condition did not improve after treatment with hormones and infliximab, and she eventually died. Positron emission tomographic-computed tomographic and B-ultrasound were performed in our hospital and showed that multiple lymph nodes were enlarged. Immunohistochemi-stry showed that CD3 and Epstein-Barr virus encoded RNA expression was positive. Colonoscopy, tissue biopsy, and histopathology showed intestinal focal mucosal infiltration of heterotypic lymphocytes with an abnormal immune phenotype. On the basis of the patient's medical history, auxiliary examination, and pathological findings, digestive physicians and pathologists gave the diagnosis of NKTCL.
Clinicians need to improve their comprehensive knowledge of NKTCL, and combination of clinical symptoms, histological characteristics, as well as colonoscopy biopsies should be considered to improve the diagnosis and thereby reduce misdiagnosis.
肠 NK/T 细胞淋巴瘤(NKTCL)发病率极低,临床症状不典型,鉴别诊断困难,易误诊为克罗恩病(CD)、T 淋巴细胞增殖性疾病等免疫相关性疾病,误诊率高,患者预后差。
本例患者反复高热,结肠镜提示多发溃疡,最初诊断为 CD,激素及英夫利昔单抗治疗后病情无好转,最终死亡。我院行正电子发射断层扫描-计算机断层扫描和 B 超检查提示多发淋巴结肿大,免疫组化示 CD3 和 Epstein-Barr 病毒编码 RNA 表达阳性。结肠镜、组织活检及病理示肠黏膜固有层局灶性异形淋巴细胞浸润,免疫表型异常。基于患者的病史、辅助检查和病理结果,消化科医师和病理科医师做出了 NKTCL 的诊断。
临床医生需要提高对 NKTCL 的综合认识,结合临床症状、组织学特征和结肠镜活检进行综合考虑,以提高诊断率,从而减少误诊。