原发肠道结外鼻型NK/T细胞淋巴瘤临床特征及生存分析

OBJECTIVE: To retrospectively analyze the clinical features and survival analysis of primary intestinal extranodal NK/T-cell lymphoma nasal type （ENKTL）. METHODS: Patients with intestinal lymphoma at the First Affiliated Hospital of Air Force Military Medical University were collected from January 2009 to December 2019, and those with primary intestinal ENKTL screened. The general situation, main symptoms, ECOG scale, lactic dehydrogenase（LDH）, and β(2)-microglobulin（β(2)-MG）in the serum, lesion site and form, numbers of extranodal invasion, Lugano stage, pathological features, and lifetime and survival outcomes were evaluated. RESULTS: In total, 34 patients with confirmed diagnosis of primary intestinal ENKTL were identified. The incidence rate of primary intestinal ENKTL is 16.2％（34/210）. A total of 26 patients were men and 8 were women with the median age at diagnosis of 45（range, 20–69）years and patients younger than 60 years account for 85.3％. It included abdominal pain（76.5％）, diarrhea（20.6％）, hematochezia（29.4％）, and abdominal mass（2.9％）, 85.3％ with B-symptoms, and abnormally elevated LDH in the serum（73.5％）. The lesion sites included large intestine（52.9％）, small intestine（29.4％）, and both of them（17.7％）. The lesion forms were featured by mass（8.8％）, ulcer（32.4％）, diffuse infiltration（2.9％）, and hybrid（55.9％）. Lugano stage included stage Ⅰ/Ⅱ（70.6％）24 patients and stage Ⅳ（29.4％）10 patients. About 94.1％ patients were EBER positive. The median survival time was 60 days. The overall survival rate had significant differences on ECOG scale, LDH in the serum, IPI score and complications （P＝0.037, 0.009, 0.002, and 0.000, respectively） CONCLUSION: Primary intestinal ENKTL was commonly observed in men at young or middle age, and the most common symptom was abdominal pain, often with B-symptoms, abnormally elevated LDH in the serum. The most common site was the large intestine. The lesion forms were mainly featured by the hybrid and ulcer. It seemed that patients with IPI low-risk group and without any complication would have longer survival time.