肠道结外鼻型NK/T细胞淋巴瘤临床特点及预后

摘要

目的：分析肠道结外鼻型NK/T细胞淋巴瘤患者的临床特点，以提高对该疾病的认识及诊疗水平。方法：回顾性分析我院曾收治的23例肠道结外鼻型NK/T细胞淋巴瘤患者的临床资料，从性别、年龄、临床症状、镜下表现、实验室检查、病理特征、治疗手段、预后情况等多方面进行分析总结。结果：男女比例2.83∶1，中位年龄45岁，中位生存期60天（95%CI：44.4~75.6），1年生存率26.1%。常见症状有发热、腹痛、腹泻、便血等，最常见部位为大肠（56.5%），其次为小肠（26.1%）、小肠＋大肠（17.4%）。内镜下肠道病变以弥漫不规则多发溃疡为主（65.2%），其次为局限性溃疡（17.4%）、黏膜病变（13.0%）、息肉样隆起（4.3%），首次肠镜＋活检确诊率17.4%。其常见并发症为穿孔、大出血。EBER阳性（P＝0.003）、急诊手术（P＝0.001）是肠道NKTCL预后不良的预测因素。结论：肠道NK/T细胞淋巴瘤发病以中青年男性为主，临床表现无特异性，易误诊。免疫组化在诊断及鉴别诊断上具有重要意义。诊断困难，预后差，穿孔或大出血前进行手术治疗可能会改善预后。临床中遇到腹痛、腹泻、便血并伴有B症状、LDH升高、EBV阳性且病情进展快的患者，应警惕NK/T细胞淋巴瘤的可能。%Objective:To analyze clinical features of intestinal extranodal NK/T cell lymphoma,nasal type,to im-prove the understanding of the disease and the diagnosis and treatment level. Methods:The clinical data of 23 intesti-nal extranodal NK/T cell lymphoma patients treated in our hospital were retrospectively analyzed for their gender,age, clinical manifestations,endoscopic findings,laboratory examination,pathological features,therapeutic approach and prognosis. Results:The male∶female ratio was 2. 83∶1,The median age was 45 years old and the median survival peri-od was 60 days(95% confidence interval:44. 4 ~75. 6). 1 -year overall survival rate was 26. 1%. The common symptoms consisted of fever,abdominal pain,diarrhea,and hematochezia. The most common site of involvement was the colorectum(56. 5%),followed by the small intestinal(26. 1%),both small and large intestinal(17. 4%). En-doscopically,the lesion types were mainly featured by multifocal or diffuse irregular ulcers(65. 2%),followed by lo-cal ulcers(17. 4%),mucosal lesion(13. 0%),tumorous lesion(4. 3%),initial accurate diagnosis rate was 17. 4%endoscopically. The common complication are intestinal perforation and intestinal massive hemorrhage. The negative prognostic factors for survival were the positive EBER(P=0. 003)and emergency operation(P=0. 001). Conclu-sion:Intestinal NK/T cell lymphoma most frequently favor young men,clinical manifestations has not specificity. Im-munohistochemistry play a key role in differential diagnosis,its diagnosis is difficult,with a poor prognosis. Surgery be-fore perforation or hemorrhage may improve prognosis. We should be alert to the possibility of NK/T cell lymphoma, when we encounter the patients who have abdominal pain,diarrhea,hematochezia and B syndrome,elevated LDH,pos-itive-EBV.