胃肠结外淋巴瘤

原发胃肠结外淋巴瘤诊疗进展1整理ppt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整理ppt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整理ppt发病率

占非霍奇金淋巴瘤的25%4整理ppt

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

Celiacdisease

睾丸

脑

T/NK鼻型

5整理pptINTERNATIONALEXTRANODALLYMPHOMASTUDY

GROUPExtranodalLymphomaSurvivalbyhistologyandsiteintheIELSGseries6整理ppt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PKochJClinOncol200115%3%75%7整理pptNonHodgkin’sLymphomaClassificationProject.Blood1997;89:3909-18Frequency%GGastricIIntestinNodalsite1.4%G-4%I0%G-25%I0%G-20%I胃肠道淋巴瘤分类MantlecellL.

DiffuselargeBcelllymphomaT-celllymphomaBurkitt.LMALTLymphoma

1%G-20%(colon)FollicularL.38%G-10%I60%8整理ppt系统检查分期MALTlymphoma:ESMOGUIDELINESDreylingM,ThieblemontC.etal.AnnOncol2021LymphomaGIlymphomaMandatoryphysicalexamcompletebloodcountsbasicbiochemicalstudies(renalandliverfunction,LDHandβ2MG,serumproteinimmunofixation)HIV,HCVandHBVserologyCTofthechest,abdomenandpelvis-GASTRIC:Gastroduodenalendoscopywithmultiplebiopsiestakenfromeachregionofthestomach,duodenum,gastro-esophagealjunctionandfromanyabnormal-appearingsite;H.pyloristatusmustbeevaluatedingastricL.

-SMALLINSTESTINE(IPSID–Immuno-ProliferativeSmallIntestinalDisease):CampylobacterJejunisearchinthetumorbiopsybyPCR,immunohistochemistryorinsituhybridizationmaybeperformed.

LARGEINTESTINE:colonoscopyRecommendedbonemarrowaspirateandbiopsyIfclinicallyindicated,head&neckMRIstudiesandotherimagingaretoberealized9整理pptAuto-antigens-ThyroidHashimotothyroiditis-SalivaryglandMyoepithelialsialoadenitis+/-SjögrenS.--LungLymphoidinterstitialpneumopathyMZL〔边缘区淋巴瘤〕:与慢性抗原刺激相关MALTLymphomasSiteInfectiousagents-StomachHelicobacterpylori-IntestinCampylobacterjejuni-OcularadnexaChlamydiapsittaci-skinBorreliaburgdorferiHepatitisCVirusMicrobialpathogens1.2.+SplenicMZLIsaacsonP,WrightDH.Cancer198310整理pptHELICOBACTERPYLORIinSTOMACHMZL:associatedwithachronicantigenicstimulation11整理pptchronicAgstimulation->chronicinflammationINFECTIONAUTOANTIGENAcquisitionofMALTAg-dependantMALTlymphomaAg-independantMALTlymphomaEpitheliumofextranodalsitesMALTCONCEPT12整理pptMALT淋巴瘤常见的遗传损伤NF-KBactivationBertoniF.etal.Oncology202113整理pptNormalstomachChronicgastritisMALTLymphoma

+additionalfactors:host,environment,geneticHPNFKBt(11;18)API2-MALT1t(1,14)BCL10t(14;18)Ig-MALT1E.DeKerviler–Saint-LouisHospital,Paris14整理ppt胃MALT淋巴瘤内镜Pseudogastritis30%Nodularinfiltration25%Ulcers45%JCDelchier–HenriMondorHospital,Créteil15整理pptNormalstomachChronicgastricMALTLymphoma

+additionalfactors:host,environment,geneticHPNFKBt(11;18)API2-MALT1t(1,14)BCL10t(14;18)Ig-MALT1ATBHussel,Lancet1993;Wootherspoon,Lancet1993;Wündisch,JCO200516整理ppt依赖H.Pylori的胃MALT淋巴瘤的治疗Hp.eradicationCompleteresponse:60%-100%Response:3to28months!Resistanceassociatedtot(11;18)Hussel,Lancet1993;Wootherspoon,Lancet1993;Wündisch,JCO2005Lymphoma17整理ppt

Reference n staging CRrate timetoCRrelapses procedure (%) (mos.) (n)

Savio,1996 12 CT 84 2-4 0 Pinotti,1997 45 CT 67 3-18 2 Neubauer,1997 50 CT±EUS 80 1-9 5 NobreLeitao,1998 17 CT+EUS 100 1-12 1 Steinbach,1999 23 CT±EUS 56 3-45 0 Montalban,2001 19 CT±EUS 95 2-19 0 Ruskone-Formestraux,2001 24 CT+EUS 79 2-18 2 LY03interimanalysis,2000190 CT 62 3-24 15抗生素和质子泵治疗stageI

胃MALT淋巴瘤18整理pptAfter5years=71%Medianfollow-up=7yearsFischbachetal,Gut56:1685-7,2007Pinottietal,10-ICMLAbstract#361StathisAetal,AnnOncol2021n=120patients抗生素治疗后的缓解期19整理pptNormalstomachChronicgastricMALTLymphomaHPt(11;18)API2-MALT1t(1,14)BCL10t(14;18)Ig-MALT1DLBCLp53deletion,p16deletion20整理pptGastricDLBCLDependanttoHp.?

10ptswithGastricDLBCL-StageIEorIIEPPI-amoxicillin-clarithromycinfor7daysCaseN°Age/sexTumorLocationStageHp.TreatmentNberoferadicationResponseTolymphomaTimetoCR(mo)1234567891067/M65/F60/M56/F44/M74/F35/M34/F75/M73/FAntrumAntrumCorpusAntrumAntrumCorpusAntrumCorpusAntrumCorpusIEIIE2IEIEIEIIE1IEIIE2IIE1IIE12111112211CRCRCRCRCRResidualMALTCR

CRCRCR1222221432JCDelchieretal.IELSG2021

Biomarkersassociatedwithantigendependance21整理pptRTinlocalizedgastricMALTlymphomaAuthor nRTdose(Gy) FFP Schechter,1998 17 28-43 100%at2yrTsang,2001 9 20-30 100%at5yr

Yahalom,2002 51 30median 89%at4yr Hitchcock,2002 9 34median 78%(100%local)GodaJS,20212525-3079%at5yr22整理ppt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整理pptLymphomaNodalExtranodalGastro-intestinalNonGastro-intestinalGastric:B-cell

MALT

DLBCL

H.Pylori

Intestinal:T-cell

Celiacdisease

Testis

Brain

T/NKnasalType

24整理ppt胃肠道弥漫大B细胞淋巴瘤60%ofprimaryGIlymphomaGIDLBCL25整理ppt临床表现侵袭性B病症大肿块坏死穿孔风险:10%!PKochJClinOncol2001:19:3861GIDLBCL26整理ppt治疗目的OneGoalTocurethepatientwiththefirstlineoftreatmentGIDLBCL27整理pptTREATMENTNosurgeryBiomarkersareneededtodetecttheHp.-dependantgastricDLBCLStandard

R-CHOPGIDLBCL28整理ppt