摘要目的 探讨肠道自然杀伤细胞(NK)/T细胞淋巴瘤的临床病理特点、诊断和鉴别诊断.方法 收集14例原发于肠道的NK/T细胞淋巴瘤的临床资料,对其形态学、免疫组织化学标记(EnVision法)情况进行观察分析并随访患者,结合相关文献进行讨论.结果 14例肠道NK/T细胞淋巴瘤男女之比为9∶5,中位年龄45岁.发生部位包括小肠6例、结肠6例、小肠和结肠同时受累2例.临床症状主要表现为腹部占位性病变或腹痛等消化道症状,可伴发热、消瘦等消耗性疾病的共同特点,严重者可并发肠穿孔或急性腹膜炎.组织学示异型的肿瘤细胞弥漫浸润肠壁全层,细胞中等大小或多形,炎性改变背景明显,常伴有围血管破坏和凝固性坏死形成.免疫表型:瘤细胞CD3ε、CD43、CD56、粒酶B、穿孔素均阳性,CD20、CD79α、髓过氧化物酶均阴性,Ki-67呈高表达.原位杂交EB病毒编码的小RNA均为核阳性.8例获得随访(0.5～36个月),其中5例在20个月内死亡.结论 肠道原发的NK/T细胞淋巴瘤是少见的结外NK/T细胞淋巴瘤,恶性度高,但因临床症状不具备特征性易导致误诊.它的正确诊断需结合临床表现和相关病史,依据病理学形态特点及免疫指标综合判断.

abstractsObjective To study the clinicopathologic features,diagnosis and differential diagnosis of intestinal natural killer (NK)/T-cell lymphoma.Methods The clinical features,histopathology,immunohistochemical findings and follow-up data of 14 cases of intestinal NK/T-cell lymphoma were retrospectively reviewed.Results The male-to-female ratio was 9∶ 5.The medium age of patients was 45 years.The sites of involvement included small intestine (6 cases),colon (6 cases) or both (2 cases).The main clinical manifestations were an abdominal mass,other gastrointestinal symptoms such as abdominal pain,as well as systemic symptoms such as fever and cachexia.Intestinal perforation complicated by acute peritonitis might occur in advanced disease.Histologically,the intestinal wall showed full-thickness infiltration by medium-sized atypical lymphoid cells with pleomorphic nuclei,prominent inflammatory background,angiocentric/angiodestructive growth pattern and coagulative necrosis.Immunohistochemical study showed that the tumor cells were positive for CD3ε,CD43,CD56,granzyme B and perforin.They were negative for CD20,CD79α and MPO.In-situ hybridization for Epstein-Barr virus encoded RNA (EBER) showed negative signals.A high proliferative index was demonstrated by Ki-67 immunostaining.Follow-up data of 8 cases were available,with duration of follow up ranging from 0.5 to 36 months.Five patients died within 20 months.Conclusions Extranodal NK/T-cell lymphoma,nasal-type primarily involving intestine is rare and tends to carry an aggressive clinical course.The relatively non-specific clinical manifestations of intestinal NK/T-cell lymphoma may result in misdiagnosis in some cases.A comprehensive evaluation of clinical manifestations,pathologic features and immunohistochemical findings is essential for definitive diagnosis.