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,NonHodgkinsLymphomaClassificationProject.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+/-SjgrenS.-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.DeKervilerSaint-LouisHospital,Paris,14,胃MALT淋巴瘤内镜,Pseudogastritis30%,Nodularinfiltration25%,Ulcers45%,JCDelchierHenriMondorHospital,Crteil,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;Wndisch,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;Wndisch,JCO2005,Lymphoma,17,ReferencenstagingCRratetimetoCRrelapsesprocedure(%)(mos.)(n)Savio,199612CT842-40Pinotti,199745CT673-182Neubauer,199750CTEUS801-95NobreLeitao,199817CT+EUS1001-121Steinbach,199923CTEUS563-450Montalban,200119CTEUS952-190Ruskone-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-/+T