胃肠结外淋巴瘤详解演示文稿

胃肠结外淋巴瘤详解演示文稿目前一页\总数二十九页\编于二十一点（优选）胃肠结外淋巴瘤目前二页\总数二十九页\编于二十一点定义

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，定义较困难目前三页\总数二十九页\编于二十一点发病率

占非霍奇金淋巴瘤的25%目前四页\总数二十九页\编于二十一点

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

目前五页\总数二十九页\编于二十一点INTERNATIONALEXTRANODALLYMPHOMASTUDY

GROUPExtranodalLymphomaSurvivalbyhistologyandsiteintheIELSGseries目前六页\总数二十九页\编于二十一点少见: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%目前七页\总数二十九页\编于二十一点NonHodgkin’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%目前八页\总数二十九页\编于二十一点系统检查分期MALTlymphoma:ESMOGUIDELINESDreylingM,ThieblemontC.etal.AnnOncol2012LymphomaGIlymphomaMandatoryphysicalexamcompletebloodcountsbasicbiochemicalstudies(renalandliverfunction,LDHandβ2MG,serumproteinimmunofixation)HIV,HCVandHBVserologyCTofthechest,abdomenandpelvis-GASTRIC:Gastroduodenalendoscopywithmultiplebiopsiestakenfromeachregionofthestomach,duodenum,gastro-esophagealjunctionandfromanyabnormal-appearingsite;H.pyloristatusmustbeevaluatedingastricL.

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

LARGEINTESTINE:colonoscopyRecommendedbonemarrowaspirateandbiopsyIfclinicallyindicated,head&neckMRIstudiesandotherimagingaretoberealized目前九页\总数二十九页\编于二十一点Auto-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.Cancer1983目前十页\总数二十九页\编于二十一点HELICOBACTERPYLORIinSTOMACHMZL:associatedwithachronicantigenicstimulation目前十一页\总数二十九页\编于二十一点chronicAgstimulation->chronicinflammationINFECTIONAUTOANTIGENAcquisitionofMALTAg-dependantMALTlymphomaAg-independantMALTlymphomaEpitheliumofextranodalsitesMALTCONCEPT目前十二页\总数二十九页\编于二十一点MALT淋巴瘤常见的遗传损伤NF-KBactivationBertoniF.etal.Oncology2011

目前十三页\总数二十九页\编于二十一点NormalstomachChronicgastritisMALTLymphoma

+additionalfactors:host,environment,geneticHPNFKBt(11;18)API2-MALT1t(1,14)BCL10t(14;18)Ig-MALT1E.DeKerviler–Saint-LouisHospital,Paris目前十四页\总数二十九页\编于二十一点胃MALT淋巴瘤内镜Pseudogastritis30%Nodularinfiltration25%Ulcers45%JCDelchier–HenriMondorHospital,Créteil目前十五页\总数二十九页\编于二十一点NormalstomachChronicgastricMALTLymphoma

+additionalfactors:host,environment,geneticHPNFKBt(11;18)API2-MALT1t(1,14)BCL10t(14;18)Ig-MALT1ATBHussel,Lancet1993;Wootherspoon,Lancet1993;Wündisch,JCO2005目前十六页\总数二十九页\编于二十一点依赖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,JCO2005Lymphoma目前十七页\总数二十九页\编于二十一点

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淋巴瘤目前十八页\总数二十九页\编于二十一点After5years=71%Medianfollow-up=7yearsFischbachetal,Gut56:1685-7,2007Pinottietal,10-ICMLAbstract#361StathisAetal,AnnOncol2009n=120patients抗生素治疗后的缓解期目前十九页\总数二十九页\编于二十一点NormalstomachChronicgastricMALTLymphomaHPt(11;18)API2-MALT1t(1,14)BCL10t(14;18)Ig-MALT1DLBCLp53deletion,p16deletion目前二十页\总数二十九页\编于二十一点GastricDLBCLDependanttoHp.?

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

CRCRCR1222221432JCDelchieretal.IELSG2011

Biomarkersassociatedwithantigendependance目前二十一页\总数二十九页\编于二十一点RTinlocalizedgastricMALTlymphomaAuthor 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,20102525-3079%at5yr目前二十二页\总数二十九页\编于二十一点烷化剂单药治疗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)目前二十三页\总数二十九页\编于二十一点LymphomaNodalExtranodalGastro-intestinalNonGastro-intestinalGastric:B-cell

MALT

DLBCL

H.Pylori

Intestinal:T-cell

Celiacdisease

Test