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原发肠道结外鼻型NK/T细胞淋巴瘤临床特征及生存分析

Clinical characteristics and survival analysis of primary intestinal extranodal NK/T-cell lymphoma, nasal type

摘要目的:回顾性研究原发肠道结外鼻型NK/T细胞淋巴瘤(ENKTL)患者的临床特征、生存及影响预后的因素。方法:收集2009年1月至2019年12月就诊于空军军医大学第一附属医院诊断为肠道淋巴瘤的病例,从中筛选原发肠道ENKTL并收集患者资料进行回顾性分析。结果:在210例肠道淋巴瘤患者中,原发肠道ENKTL患者34例(16.2%),其中男26例,女8例,中位年龄45(20~69)岁,年龄≤60岁者占85.3%。主要症状:腹痛(76.5%)、腹泻(20.6%)、便血(29.4%)、腹部包块(2.9%),85.3%伴B症状,73.5%血清LDH升高。病变部位:大肠(52.9%)、小肠(29.4%)、大肠及小肠均受累(17.7%)。内镜分型:隆起型(8.8%)、溃疡型(32.4%)、弥漫型(2.9%)和混合型(55.9%)。Lugano分期Ⅰ/Ⅱ期24例(70.6%),Ⅳ期10例(29.4%)。组织病理免疫组化EBER阳性者占94.1%。中位生存时间为60 d。ECOG评分、血清LDH、IPI评分及是否有并发症影响患者的OS率( P值分别为0.037、0.009、0.002和0.000)。 结论:原发肠道ENKTL多见于青中年男性,以腹痛最多见,常合并B症状,血清LDH多异常升高,病变多位于大肠,病灶主要呈混合型或溃疡型。IPI评分低危、无并发症者OS率较高。

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abstractsObjective: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.

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中华血液学杂志

中华血液学杂志

2020年41卷10期

818-821页

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