淋巴組織腫瘤課件

淋巴瘤

Lymphoma

基礎(chǔ)醫(yī)學院病理學系同濟醫(yī)院病理研究所敖啟林編輯pptDrJohnK.C.CHAN陳國璋編輯ppt4thEditionPublished20/9/2008編輯ppt第一節(jié)概述一、定義“來源于”成熟淋巴細胞及其前體細胞（淋巴母細胞）的惡性腫瘤，分為非霍奇金/白血病和霍奇金淋巴瘤兩大類。二、分類（一）非霍奇金淋巴瘤(NHL)：包括B，T和NK細胞（70%）

1、前體細胞：

B/T/NK前體細胞（淋巴母細胞）淋巴瘤（2%）

2、成熟細胞：成熟細胞淋巴瘤（68%）

B細胞（54%）

T細胞（12%）

NK/T或NK細胞（2%）

編輯ppt(二)霍奇金淋巴瘤(HL):特殊類型成熟B細胞淋巴瘤(30%)

結(jié)節(jié)性淋巴細胞為主型（1.5%）

經(jīng)典型(28.5%)(缺陷性B細胞)結(jié)節(jié)硬化型淋巴細胞豐富型混合細胞型淋巴細胞消減型編輯ppt第二節(jié)淋巴結(jié)的免疫結(jié)構(gòu)（immunoarchitecture）一、概念：通過免疫組織化學染色顯示淋巴結(jié)的結(jié)構(gòu)特征。包括腫瘤性或非腫瘤性細胞的成分（淋巴細胞,輔助性非淋巴細胞,淋巴竇,血管,網(wǎng)狀纖維等）、數(shù)量、分布方式等特征。通過對比，掌握正常和異常免疫結(jié)構(gòu)，對淋巴結(jié)病變進行診斷和鑒別診斷。高質(zhì)量的組織學切片和合理的免疫標記物的選擇是識別免疫結(jié)構(gòu)的關(guān)鍵。編輯ppt二、淋巴結(jié)的正常免疫結(jié)構(gòu)（一）淋巴結(jié)結(jié)構(gòu):皮質(zhì),髓質(zhì),竇,被膜和纖維小梁編輯ppt（二）B淋巴細胞分布：

1.主要在淺皮質(zhì)區(qū)的初級和次級濾泡，髓索和副皮質(zhì)區(qū)也含一定量B細胞;

2.B細胞分化過程中的形態(tài)和免疫學特征成為非霍奇金B(yǎng)細胞淋巴瘤分類的基礎(chǔ)。編輯ppt編輯ppt編輯pptPrepreBcellPreBcellImmatureBcellMatureBcellActivatedBcellPlasmacellPrecursorcells;antigennon-responsiveVirginBcells;antigenresponsiveGerminalcenter&postGCcells;antigenencounteredTdTCD20,CD22CD19,

PAX5CD79aCD10Bcl-6CD138CytoplasmicCD22編輯pptImmunoarchitectureofnormallymphnodeBcells(CD20+,CD10+,BCL-6+)Tcells(CD3+++);onlysmallnumbersofCD20+cells編輯ppt編輯ppt

1、初級濾泡：處女型B細胞和成熟小淋巴細胞；

編輯pptCD202、次級濾泡：由生發(fā)中心、套區(qū)和邊緣區(qū)構(gòu)成。編輯pptOrganizationofsecondarylymphoidfollicle

Marginalzonecells(IgM)Mantlezonecells(IgM+D)Folliclecentercells(IgM,IgG,IgAorIgE,notIgD)κ:λ約2:1，當κ:λ>8-10:1或λ:κ>3:1提示輕鏈限制表達編輯ppt次級濾泡生發(fā)中心、套區(qū)和邊緣區(qū)免疫標記：（1）生發(fā)中心(GerminalCenter,GC)：

1）中心細胞和中心母細胞：CD10+,BCL-6+,Bcl-2-,

2）濾泡樹突細胞(Folliculardendriticcells,FDC)

:CD21+,CD23+,CD35+3）T細胞：CD4+,

CD10+,CXCL13/PD-1+,少量CD57+4）可染體巨噬細胞：CD68+,lysozyme+,CD11c+（2）套區(qū):處女型B細胞和記憶細胞,表達IgM,IgD

（3）邊緣區(qū)：無特殊標記，絕大多數(shù)次級濾泡難以鑒別，腹腔淋巴結(jié)易識別。編輯pptCD20編輯pptCD79a編輯ppt編輯ppt編輯ppt編輯pptCD21編輯pptCD10Bcl-6編輯ppt反應(yīng)性濾泡增生的特點對T細胞依賴性抗原的反應(yīng)濾泡增多，大小、形態(tài)不等，可彌漫分布，主要位于皮質(zhì)區(qū)，無背靠背現(xiàn)象。典型生發(fā)中心具有極性(Polarity):明區(qū)和暗區(qū)，套區(qū)明顯，扁桃體組織更易識別；星天現(xiàn)象，中心細胞和中心母細胞混雜，免疫染色：生發(fā)中心細胞BCL-2-，ki-67指數(shù)較高。濾泡間區(qū)一般無B細胞彌漫陽性，B細胞CD43和CD5陰性。編輯ppt編輯ppt編輯pptDarkzoneLightzone編輯ppt

BCL-2

Ki67編輯pptMarginalzonecells編輯pptCD20編輯ppt（三）T淋巴細胞分布：副皮質(zhì)區(qū)(paracortex)T淋巴細胞為主(CD4>CD8):主為小淋巴細胞，偶爾出現(xiàn)大細胞(可以是活化的B細胞);含有高內(nèi)皮靜脈(Highendothelialvenules,HEV):血液淋巴細胞入淋巴結(jié)的通道;含有指狀樹突細胞(interdigitatingdendriticcells,IDC):S100+的專職抗原遞呈細胞；編輯ppt編輯ppt編輯pptProthymocyteSubcapsularthymocyteCorticalthymocyteMedullarythymocytePeripheralTcellPrecursorcellsMatureTcellsCytoplasmicSurfaceCD4CD8CD1CD4,CD8CD3CD2CD7TdT編輯ppt編輯pptIDC編輯pptCD3編輯ppt副皮質(zhì)區(qū)增生常見病因:病毒感染,接種疫苗，藥物，自身免疫疾病,皮病性淋巴結(jié)炎，腫瘤轉(zhuǎn)移等形態(tài)特征：副皮質(zhì)擴大，可形成結(jié)節(jié)；HEV增生；混合小淋巴細胞和轉(zhuǎn)化淋巴細胞，漿細胞，樹突細胞，嗜酸性粒細胞等；免疫染色：主為T細胞，或T/B細胞混合,一般無B細胞為主彌漫分布。編輯ppt編輯ppt編輯pptImmunoblastInfectiousmononucleosis編輯pptCD20CD3

LargecellscomprisemixtureofBandTcells-Reactiveprocess編輯pptCD30+cellsarescatteredandstainingisweakormoderate,whichindicatestheheterogeneity

ofthelargecellpopulation.CD30stainingisdiffuselystrongpositive,whichindicatesthemonocloneentity.

編輯pptKappaLambdaLargeBcellsarepolytypic編輯pptT細胞為主時支持良性的依據(jù)（謹慎?。。。海?）結(jié)構(gòu)不同程度保留：淋巴竇和皮質(zhì)淋巴濾泡；（2）大細胞群體分布不規(guī)則或呈地圖樣，不形成膨脹性團塊；（3）無浸潤現(xiàn)象：上皮,脂肪,血管,神經(jīng),纖維間質(zhì)中單行排列；（4）細胞無明顯異型：<2.5倍小淋巴細胞；常無透明胞質(zhì)；核形態(tài)較規(guī)則，圓形或橢圓形，輕度扭曲或折疊；（5）免疫染色:常為CD4+為主,少為CD8+為主(AIDS,骨髓移植等)；一般無CD4-CD8-或CD4+CD8+表型,PanT+（無缺失）；

少見ALK,TdT,CD56,γδTcellreceptor等表達編輯ppt

斑駁狀A(yù)utoimmunelymphoproliferativesyndrome，ALPS斑駁狀編輯pptReactivelargelymphoidcell(immunoblast)SmalllymphocytesNuclearsizeusuallydoesnotexceed2.5timesthatofsmalllymphocyteNucleususuallyroundorovoid,anddoesnotshowirregularfoldings編輯ppt（四）髓索（medullarycord）富含漿細胞可以清晰或不清晰反應(yīng)性B細胞的擴張延伸，如反應(yīng)性淋巴濾泡增生，漿細胞型Castlemandisease。編輯ppt編輯ppt編輯ppt（五）淋巴竇（sinus）輸入淋巴管被膜下竇間竇髓竇輸出淋巴管；被覆竇內(nèi)皮(D2-40),含組織細胞和淋巴樣細胞；腹腔內(nèi)淋巴結(jié)竇常擴張，較明顯；深處竇的存在常提示良性病變。編輯ppt編輯ppt編輯ppt編輯ppt編輯ppt（六）纖維結(jié)締組織網(wǎng)

Connectivetissueframework纖維被膜,延伸至結(jié)內(nèi)的梁索(可見梁索周竇)；正常時可能不清晰；反應(yīng)性病變常清晰，常有淋巴結(jié)周圍炎。編輯ppt編輯ppt編輯ppt第三節(jié)淋巴瘤分類

WHOClassification2008

非霍奇金淋巴瘤

non-Hodgkinlymphomas

霍奇金淋巴瘤

Hodgkinlymphomas編輯ppt非霍奇金淋巴瘤分類

WHOClassificationof

non-Hodgkinlymphomas(2001)Precursorlymphoblasticlymphoma/leukemia(BorTcelltype)MatureB-cellneoplasmsMatureT-cellandNK-celllymphomas編輯ppt成熟B細胞腫瘤

MatureB-cellneoplasms(WHO)B-cellCLL/SLLB-cellprolymphocyticleukemiaLymphoplasmacyticlymphomaMantlecelllymphomaFollicularlymphomaExtranodalmarginalzoneB-celllymphomaofMALTtypeNodalmarginalzoneB-celllymphomaSplenicmarginalzoneBcelllymphomaHairycellleukemiaDiffuselargeB-celllymphoma(includingmediastinallargeB-celllymphoma)BurkittlymphomaPlasmacytoma,myeloma編輯ppt成熟T細胞或NK細胞腫瘤

MatureT-cell&NK-cellneoplasms(WHO)T-cellprolymphocyticleukemiaT-celllargegranularlymphocyticleukemiaAggressiveNKcellleukemiaExtranodalNK/Tcelllymphoma,nasal-typeMycosisfungoides,SezarysyndromeAngioimmunoblasticTcelllymphomaPeripheralT-celllymphoma,unspecifiedAdultT-cellleukemia/lymphomaAnaplasticlargecelllymphoma(Tornullcell),primarysystemictypePrimarycutaneousanaplasticlargecelllymphomaSubcutaneouspanniculitis-likeT-celllymphomaEnteropathy-typeT-celllymphomaHepatosplenicT-celllymphoma編輯ppt非霍奇金淋巴瘤臨床生物學行為分類Indolentlymphoma(“Good”)Aggressivelymphoma

(“Bad”)Highlyaggressivelymphoma(“Ugly”)Unusuallocalizedindolentlymphoma編輯ppt惰性淋巴瘤

Indolentlymphomas:

smallcelllymphoma

FollicularlymphomaB-cellCLL/smalllymphocyticlymphomaLymphoplasmacyticlymphomaSplenicandnodalmarginalzonelymphoma編輯ppt惰性淋巴瘤臨床特征UsuallyolderadultsSlow-growing;occasionallywaxingandwaningUsuallyhighstage(III/IV)atpresentation;blood&marrowinvolvementcommonTakesmanyyearstokillpatientifuntreatedLowproliferativefraction:oftenincurable.Despitetreatment(diseasecontrol),repeatedrelapseistheruleTreatmentdoesnotaltertheeventualoutcome,thusobservationwithouttreatmentisanoption編輯pptSurvivalcurveofindolentlymphomas%Survival100%0%Years010編輯ppt侵襲性淋巴瘤

Aggressivelymphomas:

largecelllymphoma

DiffuselargeB-celllymphomaMantlecelllymphoma*

AllperipheralTcelllymphomas(includingALCL&NKcelllymphomasexcept:Mycosisfungoides)PrimarycutaneousanaplasticlargecelllymphomaT-cellLGLleukemia編輯ppt侵襲性淋巴瘤臨床特征Anyage,usuallyadultsRapid-growingmassEvenlydistributedoverthestages;blood/marrowinvolvementuncommon(badprognosis;notsobadifmarrowinvolvedbyfollicularlymphoma)Untreated,willkillin1-2yearsHighproliferativefraction:potentiallycurablewithchemotherapy+/-radiotherapy80%CR;2/3norelapse編輯pptSurvivalcurveofaggressivelymphomas%Survival100%0%Years010TreatedUntreated編輯pptComparisonofsurvivalcurves

%Survival100%0%Years010AggressiveNHLIndolentNHLParadoxicallybetteroutcomeinthelongrun編輯ppt高度侵襲性淋巴瘤

Highlyaggressivelymphomas:

Medium-sizedcelllymphoma

Lymphoblasticlymphoma

Burkittlymphoma編輯pptHighlyaggressivelymphomasChildren,youngadultsVeryrapidlygrowing:oncologicemergencyOftenhighstage;marrowinvolvementcommon;CNSinvolvementmayoccurUntreated,willkillinweekstomonthsVeryhighproliferativefraction:highlyresponsivetotreatment.Standardchemotherapy:nogood;veryaggressivechemotherapy:manycured編輯pptSurvivalcurveofhighlyaggressivelymphomas%Survival100%0%Years010TreatedUntreated編輯ppt局限性惰性淋巴瘤

LocalizedindolentlymphomasExtranodalmarginalzoneB-celllymphomaofmucosa-associatedlymphoidtissue

（MALT）

typePrimarycutaneousanaplasticlargecelllymphoma編輯pptLocalizedindolentlymphomasIndolenttumorCanoccurinanyageOftenlocalizedatpresentationManypatientscanapparentlybecured(thereisasuggestionofplateauinsurvivalcurve),althoughlaterelapsesarepossibleCanundergoregressionspontaneousforprimarycutaneousALCLwithanti-HelicobactertherapyforgastricextranodalMZL編輯ppt各型淋巴瘤的臨床病理特征編輯ppt淋巴母細胞淋巴瘤RapidlygrowingtumorLymphoma:T>>BLeukemia(ALL):B>>TUntreated:rapidlyfatalAggressivetherapy:goodsurvivalCannotpredictlineagefrommorphologyDefiningmarker:TdT+，CD99編輯ppt編輯ppt編輯pptCD20CD79aB-LBL編輯pptT-LBL編輯ppt濾泡性淋巴瘤

FOLLICULARLYMPHOMAUsuallyinvolvinglymphnodesHigh-stagediseaseatpresentationPathology:RecapitulatestheorganizationandcytologyofthenormalgerminalcentersKeyimmunophenotype:Bmarker+,CD10+,Bcl6+,Bcl2+Follicularlymphomainvolvingsomeextranodalsites(suchasskin,duodenum,testis)showdistinctiveclinicalandbiologicfeaturesGenetics:Iggenerearrangement;

t(14;18)(q32;q21)

bcl-2generearrangement

(duetoBCL2/IGHtranslocation)編輯pptFollicularlymphomavs

reactivefollicularhyperplasiaMajorcriterionCloselypackedfolliclesthroughout,withscantyinterfolliculartissues1majorcriterionor3minorcriteria.Butconfirmbyancillarytestsifuncertain編輯ppt濾泡性淋巴瘤主要診斷標準示意圖

Majorcriterionfulfilledin80%ofcasesoffollicularlymphomaJustgoontohighmagnificationtoconfirmfolliclecentercellmorphology編輯ppt編輯ppt編輯pptCentrocytesoftenhavetriangularshapednuclei編輯ppt濾泡性淋巴瘤的次要診斷標準Intheremaining20%ofcases(non-crowdedfollicles),acombinationofminorcriteria(3)

havetobeconsidered,andsupplementedbyancillarystudiesasrequired編輯pptMinorcriteria

Notingible-bodymacrophagesCellularmonotony:centrocytepredominanceNocellularpolarisationAbsentorincompletemantlesFolliclesinperinodaltissuesDysplasticfollicularcentercells,e.g.signetringcells,frequentcytoplasmicglobulesAtypicalcellsininterfolliculartissuesInvasionofwallsofbloodvessels編輯ppt編輯ppt編輯ppt編輯ppt編輯pptFollicularlymphomavsRFH:ancillarytechniquesImmunostainingforbcl-2MostusefulimmunostainNormal:BothTandBcellspositiveexceptfolliclecentercellsLookforpositivestainingofcellsinfolliclecenters:positivestainingsupportsdiagnosisoflymphoma編輯pptReactivefollicles編輯pptFollicularlymphoma(Bcl-2immunostaining)編輯pptCommonmisconceptionsinhematopathology"Negativebcl-2stainrulesoutfollicularlymphoma"Thetruth:Itdoesnot.20-25%offollicularlymphomacasesarenegative.Pursuewithotherteststoconfirmorrefuteadiagnosisoffollicularlymphoma編輯pptBcl-2stainingin

follicularlymphoma編輯ppt編輯pptBcl-2編輯pptLookforevidenceinterfollicularinvasionDenselypackedCD20+orCD79+B-cellsbetweenfolliclesisindicativeofinterfollicularinvasionPresenceofasignificantnumberofCD10+lymphoidcells(notneutrophils,whicharealsoCD10+)betweenfolliclesalsoindicatesinterfollicularinvasionLookforvascularinvasion

ManyCD10+cellsinthewallsofperinodalbloodvessels編輯pptCD20編輯pptCD10編輯ppt編輯pptLookformonoclonalityorlowproliferationindexImmunostainforIgtolookforlightchainrestrictionDemonstratedbyKi67,alowKi67indexfavorsdiagnosisoffollicularlymphomaoverRFH(meanindex15.6%vs64.9%);ahighKi67indexisinconclusiveMolecularanalysisIggenerearrangementbySouthernblotorPCR(NotethatPCRmayhavefalsenegativeresults)DetectionofBCL2generearrangementbySouthernblot,PCRorFISH編輯pptKappaLambda編輯pptFL1:Ki67RFH:Ki67編輯pptFL3:Ki67編輯ppt套細胞淋巴瘤

Mantlecelllymphoma:morphologyDiffuse,vaguelynodular,ormantlezonepatternBroadcytologicspectrumClassictype:smalllymphoidcellswithirregulartoroundnucleiBlastoidvariant(~lymphoblasts,butTdT-)PleomorphicvariantOftenwithsolitaryepithelioidhistiocytesInterspersed‘nakednuclei’(folliculardendriticcells)Genetics:Iggenerearrangementt(11,14)(q13;q32)編輯pptGrowthpatternofMCL編輯ppt編輯ppt編輯ppt編輯pptRoundnuclei編輯pptMantlecelllymphoma:

biologicmarkersBmarker+,CD5+,CD10-,CD23-,IgM+,IgD+Keydefiningfeature:CyclinD1+dueto

t(11;14)(q13;q32)

orcryptictranslocation編輯pptCyclinD1編輯ppt

粘膜相關(guān)淋巴組織

Mucosa-AssociatedLymphoidTissue(MALT)SpecializedsecondarylymphoidcompartmentsofmucosaltissuesNatural,e.g.,Waldeyer’sring,smallintestine,lungAcquiredinautoimmunedisease/inflammatione.g.,thyroid,salivarygland,lacrimalgland,stomachSimilarorganizationaslymphnode:B-cellfollicles,T-cellzonesLymphoidcellsacquirehomingproperties編輯ppt編輯pptLOWGRADEBCELLMALTLYMPHOMA(extranodalmarginalzoneB-celllymphoma)Usuallylocalizedatpresentation(66-79%)RecurrenceatotherMALTsites(“homing”)Manycanbetreatedbylocoregionaltherapyoreradicationofantigen-stimulationsourceCompleteremissionrate=72%5-yearoverallsurvival=74%(Gastric:90%)編輯pptHistologicfeaturesoflowgrade

MALTlymphomaSmall/medium-sizedcells(lymphocytes,centrocyte-like,monocytoidBcells)Patterns:diffuse,interfollicular,marginalzoneLymphoepitheliallesionsPlasmacellsarecommonReactivelymphoidfollicles+/-colonization編輯ppt編輯ppt編輯ppt編輯pptLowgradeMALTlymphoma:ImmunophenotypeAneoplasmofpost-germinalcenterBcellsPanB+,CD10-,CD5-,CyclinD1-,CD23-Keyfeatures:siteofinvolvementlymphoepitheliallesionexclusionofotherlowgradeB-celllymphomas編輯pptAfterHelicobactereradicationtherapy編輯ppt彌漫性大B細胞淋巴瘤

DIFFUSELARGEBCELLLYMPHOMA（DLBCL）CommonestnodalB-celllymphomaAheterogeneousentityFinalcommonpathwayforhighgradetransformationoflowgradeB-celllymphomas編輯pptCentroblastic/Immunoblastic編輯ppt編輯pptMultilobatedPleomorphic編輯pptBURKITTLYMPHOMAThreedifferentforms:Endemic(EquatorialAfrica;strongassociationwithEBV>90%;commonlyaffectingjaws,gonads,kidneys)Sporadic(EBVassociationonly~15%;commonlyinvolvingterminalileumandWaldeyer’sring)AIDS-related(EBVassociation~30%;commonlyinvolvinglymphonodeandbonemarrow)AfricancasescanalsobeAIDS-related!編輯pptBurkittlymphomat(8;14)(q24;q32)orvariants,withtranslocationbetweenc-mycandIggeneMostlyadiseaseofyoungpatientsHighlyaggressive,butpotentiallycurablebychemotherapy編輯ppt編輯ppt編輯pptBurkittlymphoma:ImmunogeneticsProbablycorrespondstoIgM+blastsingerminalcenterPan-B+,CD10+,Bcl-6+,Bcl-2-Keyfeatures:C-MYCtranslocationmedium-sizedcellshighKi67index：～100%germinalcentercellphenotype編輯pptCD20CD10編輯pptKi67編輯ppt外周T和NK細胞淋巴瘤

PERIPHERALTANDNKCELLLYMPHOMASUncommon;<10%ofallnon-Hodgkin’slymphomasinCaucasians,but15-20%inChineseLessfavorableoutcomecomparedwithaggressiveBcelllymphomas編輯pptTandNKcelllymphomas:

EntitieswithdifferentoutcomeFavorableprognosisPrimarycutaneousanaplasticlargecelllymphoma(+/-spontaneousregression)Anaplasticlargecelllymphoma,T/nullcellMycosisfungoidesT-cellLGLleukemiaHighlyaggressiveAdultT-cellleukemia/lymphomaNKcellleukemiaExtranodalNK/TcelllymphomaHepatosplenic

Tcelllymphoma編輯pptPeripheralTcelllymphoma

外周T細胞淋巴瘤PredominantlynodalAngioimmunoblasticT-celllymphomaAnaplasticlargecelllymphoma,T/null-cellPeripheralT-celllymphoma,unspecified編輯pptANGIOIMMUNOBLASTIC

T-CELLLYMPHOMA

血管免疫母細胞性T細胞淋巴瘤CommonesttypeofnodalTcelllymphomaFever,skinrash,systemicsymptomsGeneralizedlymphnodesIncreasedserumIg(polyclonal);CoombspositivehemolyticanemiaAlthoughsomerespondtosteroid,prognosisisunfavorable;succumbingtoinfectioncomplications編輯pptAneoplasmoffollicularcenterThelpercellsPanT+,usuallyCD4+(butalsomanyCD8+cells),CD10+,CXCL13+,PD-1Keyfeatures:ArborizingvenulesLymphoidcellsoftenwithroundnuclei;clearcellscommonirregularmeshworksoffolliculardendriticcellaroundvenulesCD10/CXCL13+TcellsoutsidefollicleIsolatedEBV+cells(mostlyBcells)編輯ppt編輯ppt編輯pptCD3CD21編輯pptCXCL13編輯pptEXTRANODALNK/TCELLLYMPHOMA

結(jié)外NK/T細胞淋巴瘤Mass-formingneoplasmofputativeNKcelllineage(cCD3+,sCD3-,CD56+,germlineTCRgenes)Designated“NK/Tcell”becausewhilemostcasesappeartobeofNKlineage,somemaybecytotoxicTcellneoplasms編輯pptMoreprevalentamongOrientals,MexicansandSouthAmericansthanWesternpopulationsMostlyaffectingadultsMostlyinvolvingextranodalsites編輯pptExtranodalNK/TcelllymphomaNASALNK/TCELLLYMPHOMAInvolvingupperaerodigestivetractNasalobstruction(masslesion)ormidfacialdestructivediseaseUsuallystageIBehaviorvariablebutgenerallyaggressiveEXTRANASALNK/TCELLLYMPHOMAInvolvingvariousextranasalsites(esp.skin,testis,G.I.tract,softtissue)UsuallystageIII/IVHighlyaggressive,withmostdyingwithin1-2years(>80%)編輯pptExtranodalNK/Tcelllymphoma:PathologyNecrosis(apoptosis)commonAngioinvasioncommon(notinvariablyseen)Broadcytologicspectrum:small,medium-sizedorlargecells,ormixedMayhavemanyadmixedinflammatorycells編輯ppt編輯ppt編輯pptSmallMedium-sizedLargeBroadcytologicspectrum編輯pptExtranodalNK/Tcelllymphoma:

immunophenotypeCD2+.sCD3-,cCD3e+,CD56+

Cytotoxicgranulesperforin,granzymeB,TIA-1positiveCD4,CD5,CD7,CD8:usuallynegativeKeyfeatures:CD56+,EBER+IfCD56-,requiresEBER+andcytotoxicgranules+fordiagnosis編輯pptSurfaceCD3CytoplasmicCD3εCD56FrozentissueParaffinsection編輯pptPERIPHERALTCELLLYMPHOMAUNSPECIFIED

外周T細胞淋巴瘤，非特殊型AwastebasketcategoryVariableclinicalmanifestationsUsuallyhighstagedisease(nodal/extranodal)MayhaveeosinophiliaAggressive;relapsecommonTheextranodalcasesoftenexpressacytotoxiccellphenotype編輯pptPeripheralT-celllymphoma:

MorphologiccluesforrecognitionPredominantparacorticalinvolvementProminenthighendothelialvenulesSpectrumofcellssizesandshapesIrregularnuclearcontoursClearcellsManyadmixedepithelioidhistiocytesandeosinophilsButimmunostainingessentialforconfirmingTlineage編輯ppt編輯ppt編輯pptANAPLASTICLARGECELLLYMPHOMA,primarysystemicform

間變性大細胞淋巴瘤，原發(fā)系統(tǒng)性TendstooccurinchildrenandyoungadultsNodalorextranodalpresentation(whichmayincludeskin,softtissue,bone,G.I.tract)Immunophenotype:CD30+;EMAusually+T-lineagemarkers+/-(Tornullcell)AproportionofcasesexpressALKduetotranslocationinvolvingALKgene編輯pptHallmarkcells編輯pptALKtranslocation(orexpression)hashelpedtodefineahomogeneoussubgroupofALCLThiscanbeeasilydetectedbyimmunostainingforALK--permittingre-definitionofthemorphologicspectrumofALCL:ClassicaltypeMonomorphicSmallcellLymphohistiocyticHypocellular編輯ppt編輯pptCD30編輯ppt

Overallsurvival

PrimarysystemicformofALCL

0510YearsALCL,ALK+*100%50%ALCL,ALK-編輯ppt

霍奇金淋巴瘤

HODGKINLYMPHOMA編輯pptLymphocytepredominanceMixedcellularityNodularsclerosisLymphocytedepletionNodularlymphocytepredominantHodgkinlymphoma

(N-LPHL)ClassicalHodgkinlymphoma(cHL)Lymphocyte-richMixedcellularityNodularsclerosisLymphocytedepletionRY