胃肠结外淋巴瘤ppt课件

原发胃肠结外淋巴瘤诊疗进展 1 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 2 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 3 发病率 占非霍奇金淋巴瘤的25 4 淋巴瘤 淋巴结 淋巴结外 胃肠道 非胃肠道 胃 B cellMALTDLBCLH Pylori 肠道 T cellCeliacdisease 睾丸脑T NK鼻型 5 INTERNATIONALEXTRANODALLYMPHOMASTUDYGROUP ExtranodalLymphomaSurvivalbyhistologyandsiteintheIELSGseries 6 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 PKochJClinOncol2001 15 3 75 7 NonHodgkin sLymphomaClassificationProject Blood1997 89 3909 18 Frequency GGastricIIntestinNodalsite 1 4 G 4 I 0 G 25 I 0 G 20 I 胃肠道淋巴瘤分类 MantlecellL DiffuselargeBcelllymphoma T celllymphoma Burkitt L MALTLymphoma 1 G 20 colon FollicularL 38 G 10 I 60 8 系统检查分期 MALTlymphoma ESMOGUIDELINESDreylingM ThieblemontC etal AnnOncol2012 9 Auto antigens ThyroidHashimotothyroiditis SalivaryglandMyoepithelialsialoadenitis Sj grenS LungLymphoidinterstitialpneumopathy MZL 边缘区淋巴瘤 与慢性抗原刺激相关 MALTLymphomas SiteInfectiousagents StomachHelicobacterpylori IntestinCampylobacterjejuni OcularadnexaChlamydiapsittaci skinBorreliaburgdorferi HepatitisCVirus Microbialpathogens 1 2 SplenicMZL IsaacsonP WrightDH Cancer1983 10 HELICOBACTERPYLORIinSTOMACH MZL associatedwithachronicantigenicstimulation 11 chronicAgstimulation chronicinflammation MALTCONCEPT 12 MALT淋巴瘤常见的遗传损伤 NF KBactivation BertoniF etal Oncology2011 13 Normalstomach ChronicgastritisMALTLymphoma additionalfactors host environment genetic HP NFKB t 11 18 API2 MALT1t 1 14 BCL10t 14 18 Ig MALT1 E DeKerviler Saint LouisHospital Paris 14 胃MALT淋巴瘤内镜 Pseudogastritis30 Nodularinfiltration25 Ulcers45 JCDelchier HenriMondorHospital Cr teil 15 Normalstomach ChronicgastricMALTLymphoma additionalfactors host environment genetic HP NFKB t 11 18 API2 MALT1t 1 14 BCL10t 14 18 Ig MALT1 ATB Hussel Lancet1993 Wootherspoon Lancet1993 W ndisch JCO2005 16 依赖H Pylori的胃MALT淋巴瘤的治疗 Hp eradication Completeresponse 60 100 Response 3to28months Resistanceassociatedtot 11 18 Hussel Lancet1993 Wootherspoon Lancet1993 W ndisch JCO2005 Lymphoma 17 ReferencenstagingCRratetimetoCRrelapsesprocedure mos n Savio 199612CT842 40Pinotti 199745CT673 182Neubauer 199750CT EUS801 95NobreLeitao 199817CT EUS1001 121Steinbach 199923CT EUS563 450Montalban 200119CT EUS952 190Ruskone Formestraux 200124CT EUS792 182LY03interimanalysis 2000190CT623 2415 抗生素和质子泵治疗stageI胃MALT淋巴瘤 18 After5years 71 Medianfollow up 7years Fischbachetal Gut56 1685 7 2007Pinottietal 10 ICMLAbstract 361StathisAetal AnnOncol2009 n 120patients 抗生素治疗后的缓解期 19 Normalstomach ChronicgastricMALTLymphoma HP t 11 18 API2 MALT1t 1 14 BCL10t 14 18 Ig MALT1 DLBCL p53deletion p16deletion 20 GastricDLBCLDependanttoHp 10ptswithGastricDLBCL StageIEorIIEPPI amoxicillin clarithromycinfor7days JCDelchieretal IELSG2011 Biomarkersassociatedwithantigendependance 21 RTinlocalizedgastricMALTlymphoma AuthornRTdose Gy FFPSchechter 19981728 43100 at2yrTsang 2001920 30100 at5yrYahalom 20025130median89 at4yrHitchcock 2002934median78 100 local GodaJS 20102525 3079 at5yr 22 烷化剂单药治疗 24例患者 17例stageI 7例stageIVCyclophosphamideorChlorambucilfor8 24mos 100 ORR 75 CR 5 yearEFS 50 5 yearOS 75 5relapsesatinitialsites 1withtransformation Hammeletal JCO1995 cyclophosphamideorchlorambucil 23 Lymphoma Nodal Extranodal Gastro intestinal NonGastro intestinal Gastric B cellMALTDLBCLH Pylori Intestinal T cellCeliacdisease TestisBrainT NKnasalType 24 胃肠道弥漫大B细胞淋巴瘤 60 ofprimaryGIlymphoma GIDLBCL 25 临床表现 侵袭性B症状大肿块坏死穿孔风险 10 PKochJClinOncol2001 19 3861 GIDLBCL 26 治疗目的 OneGoal Tocurethepatientwiththefirstlineoftreatment GIDLBCL 27 TREATMENT NosurgeryBiomarkersareneededtodetecttheHp dependantgastricDLBCLStandardR CHOP GIDLBCL 28 Lymphoma Nodal Extranodal Gastro intestinal NonGastro intestinal Gastric B cellMALTDLBCLH Pylori Intestinal T cellCeliacdisease TestisBrainT NKnasalType EATL 29 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 RelativeriskofEATLx50 100临床表现 多发空肠溃疡Extension GItract estomac colonExtra intestinal blood skin lung EATL 30 EATL 粘膜内T淋巴细胞增殖PhenotypeTCD3 CD4 CD8 TCR CD103 PhenotypeTcytotoxicTIA1 granzymeB GranzymeB CD103 组织学表现 31 治疗 不含谷蛋白膳食NogoodchemotherapyOSpoor1year 35 5years 20 EATL 32 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 33 Thanksforyourattention 34