原发胃肠结外淋巴瘤诊疗进展 刘艳艳.pptVIP

肠病型小肠T细胞淋巴瘤(EATL) 通常有谷蛋白敏感性肠病既往史 腹腔疾病患者: Relative risk of EATL x 50-100 临床表现: 多发空肠溃疡 Extension: GI tract : estomac, colon Extra-intestinal : blood, skin, lung EATL EATL 粘膜内T淋巴细胞增殖 Phenotype T CD3+CD4- CD8-/+ TCR- CD103+ Phenotype T cytotoxic TIA1+ granzyme B+ Granzyme B CD103 组织学表现 治疗 不含谷蛋白膳食 No good chemotherapy OS poor 1 year = 35% 5 years = 20% EATL 结论 结外淋巴瘤具有独特的临床病理特征 胃肠道淋巴瘤最常见 多数与慢性抗原刺激相关 治疗应采取个体化 * * 20 * * * MZL and particularly MALT lymphoma is believed to be an antigen-dependent disease. Persistent antigenic challenge causes a chronic inflammatory reaction wich can in some people develop into more organized MALT with histologic similarity to peyer’s patches in the small intestine. * * Either infection or autoantigen will induce on the epithelium of extranodal sites, a chronic inflammation with stroma remodelling, angiogenesis This will induce a proliferation of B cells through te recruitment and the activation of T-cell, and a MALT will be acquired Genetic lesions will occur because of ROS production The result will be an Ag-dependent MALT lymphoma Additional genetic lesions will eventually make the lymphoproliferation independent of antigenic stimulation * * MALT lymphomas present with a series of recurrent genomic lesions, including chromosomal translocations and unbalanced genomic aberrations * * * * * * * * * * * * 原发胃肠结外淋巴瘤诊疗进展 刘艳艳 河南省肿瘤医院淋巴综合内科 P-MAR-2015.06-046 Valid Until 2017.06 专业资料，仅供医药卫生专业人士使用 声明 本幻灯片代表个人观点。处方请参考国家食品药品监督管理总局批准的药物说明书。 定义 来源于淋巴结外的淋巴组织 甚至来源于正常情况下不含淋巴组织的部位 当结内和结外病变同时存在时，定义较困难 发病率 占非霍奇金淋巴瘤的25% 淋巴瘤 淋巴结 淋巴结外 胃肠道 非胃肠道 胃 : B-cell MALT DLBCL H. Pylori 肠道 : T-cell Celiac disease 睾丸脑T/NK 鼻型 INTERNATIONAL EXTRANODAL LYMPHOMA STUDY?GROUP Extranodal Lymphoma Survival by histology and site in the IELSG series 少见 : 所有胃肠道肿瘤的3% 绝大多数胃肠道淋巴瘤来源于胃 原发胃肠道淋巴瘤 P Koch J Clin Oncol 2001 15% 3% 75% Non Hodgkin’s Lymphoma Classification Project. Blood 1997;89:3909-18 Frequency % G