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词汇介绍
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解析
enteropathy [,entə'rɒpəθɪ]
释义 n. 肠道病
例句 Diabetes enteropathy is diabetes one of many complications, the modern medicine still not was too at present clear to this sickness pathogenesis, also lacks the effective method in the treatment. 糖尿病肠病是糖尿病诸多并发症之一,现代医学对本病发病机制尚不太清楚,在治疗上也缺乏行之有效的方法。
associated 英[ə'səuʃi,eitid] 美 [əˈsoʊsieɪtɪd,əˈsoʊʃieɪtɪd]
释义 adj. 关联的;联合的
v. 联系(associate的过去式和过去分词)
例句 Ventilator-associated pneumonia(VAP)causes substantial morbidity. 呼吸机相关性肺炎(VAP)会引起重大的死亡率。
cell 英 [sel] 美 [sel]
释义 n. 细胞;电池;蜂房的巢室;单人小室
vi. 住在牢房或小室中
例句 You can move only one ball to any empty cell. 你只能移动一个球到任何空的单元格。
lymphoma 英 [lɪmˈfəʊmə] 美 [lɪmˈfoʊmə]
释义 n. [肿瘤] 淋巴瘤
复数 lymphomas或lymphomata
例句 The Leukemia & Lymphoma Society can also direct you to resources to assist you in talking with your children . 白血病及淋巴瘤协会也可以为你提供一些方法,帮助你和孩子进行交流。
概述
概述
肠病型T细胞淋巴瘤(enteropathy-associated T-cell lymphoma,EATL)是一种来源于上皮间T淋巴细胞的肿瘤,属于非霍奇金淋巴瘤的一个亚型,即结外淋巴瘤,该病最常发生在空肠、回肠,极少见于胃、十二指肠、结肠及消化道以外的部位,可呈节段性分布,表现为多灶性不规则溃疡。
病因及发病机制
该病致病因素未完全阐明,目前认为和染色体异常、感染、免疫系统异常及其他理化学因素有关。
临床表现
EATL最常见的临床表现为腹痛进行性加重,体重下降和难以控制的发热,以腹痛、体质量下降、发热、腹块、便血和大便习惯改变为主要临床表现,尤以肠道多发溃疡伴发热对诊断有提示作用。可伴有腹泻、便血、营养不良,少数见腹块、消化道梗阻、肝脾肿大和全血细胞减少等症状。晚期患者可出现肠穿孔,一旦发生穿孔,病情将迅速恶化。
诊断与鉴别诊断
诊断方面,无特异性临床表现。常用辅助检查方式有CT/MRI、18-氟脱氧葡萄糖PET成像、内镜检查、免疫组化鉴定、PCR技术,在临床诊断中,强调PCR检测结果分析与光镜下组织学改变、免疫组化相结合进行诊断。但其确诊必须依靠病理检查。
鉴别诊断方面,EATL需与下列疾病相鉴别:
(1)肠黏膜相关淋巴瘤;
(2)肠白塞病;
(3)肠结核;
(4)结肠癌;
(5)克罗恩病
治疗
对于EATL患者,要尽早化疗,若出现肠穿孔、肠梗阻等并发症时要及时行手术治疗。手术是通过切除肿瘤病灶,减轻肠梗阻症状,或切除穿孔、坏死组织,以治疗并发症。手术并不能完成根治性治疗。国外有研究表明自体外周血干细胞移植可有效改善预后,延缓病程。
Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2复制标题
肠病相关T细胞淋巴瘤亚型以SETD2功能丧失为特征
发表时间:2017-05-01
影响指数:10.9
作者: Andrea B. Moffitt
期刊:The Journal of Experimental Medicine
Enteropathy-associated T cell lymphoma (EATL) is a lethal, and the most common, neoplastic complication of celiac disease. Here, we defined the genetic landscape of EATL through whole-exome sequencing of 69 EATL tumors. SETD2 was the most frequently silenced gene in EATL (32% of cases). The JAK-STAT pathway was the most frequently mutated pathway, with frequent mutations in STAT5B as well as JAK1, JAK3, STAT3, and SOCS1 We also identified mutations in KRAS, TP53, and TERT Type I EATL and type II EATL (monomorphic epitheliotropic intestinal T cell lymphoma) had highly overlapping genetic alterations indicating shared mechanisms underlying their pathogenesis. We modeled the effects of SETD2 loss in vivo by developing a T cell-specific knockout mouse. These mice manifested an expansion of γδ T cells, indicating novel roles for SETD2 in T cell development and lymphomagenesis. Our data render the most comprehensive genetic portrait yet of this uncommon but lethal disease and may inform future classification schemes.
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