淋巴组织肿瘤课件

淋巴瘤

Lymphoma

基础医学院病理学系同济医院病理研究所敖启林编辑pptDrJohnK.C.CHAN陈国璋编辑ppt4thEditionPublished20/9/2008编辑ppt第一节概述一、定义“来源于”成熟淋巴细胞及其前体细胞（淋巴母细胞）的恶性肿瘤，分为非霍奇金/白血病和霍奇金淋巴瘤两大类。二、分类（一）非霍奇金淋巴瘤(NHL)：包括B，T和NK细胞（70%）

1、前体细胞：

B/T/NK前体细胞（淋巴母细胞）淋巴瘤（2%）

2、成熟细胞：成熟细胞淋巴瘤（68%）

B细胞（54%）

T细胞（12%）

NK/T或NK细胞（2%）

编辑ppt(二)霍奇金淋巴瘤(HL):特殊类型成熟B细胞淋巴瘤(30%)

结节性淋巴细胞为主型（1.5%）

经典型(28.5%)(缺陷性B细胞)结节硬化型淋巴细胞丰富型混合细胞型淋巴细胞消减型编辑ppt第二节淋巴结的免疫结构（immunoarchitecture）一、概念：通过免疫组织化学染色显示淋巴结的结构特征。包括肿瘤性或非肿瘤性细胞的成分（淋巴细胞,辅助性非淋巴细胞,淋巴窦,血管,网状纤维等）、数量、分布方式等特征。通过对比，掌握正常和异常免疫结构，对淋巴结病变进行诊断和鉴别诊断。高质量的组织学切片和合理的免疫标记物的选择是识别免疫结构的关键。编辑ppt二、淋巴结的正常免疫结构（一）淋巴结结构:皮质,髓质,窦,被膜和纤维小梁编辑ppt（二）B淋巴细胞分布：

1.主要在浅皮质区的初级和次级滤泡，髓索和副皮质区也含一定量B细胞;

2.B细胞分化过程中的形态和免疫学特征成为非霍奇金B细胞淋巴瘤分类的基础。编辑ppt编辑ppt编辑pptPrepreBcellPreBcellImmatureBcellMatureBcellActivatedBcellPlasmacellPrecursorcells;antigennon-responsiveVirginBcells;antigenresponsiveGerminalcenter&postGCcells;antigenencounteredTdTCD20,CD22CD19,

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

1、初级滤泡：处女型B细胞和成熟小淋巴细胞；

编辑pptCD202、次级滤泡：由生发中心、套区和边缘区构成。编辑pptOrganizationofsecondarylymphoidfollicle

Marginalzonecells(IgM)Mantlezonecells(IgM+D)Folliclecentercells(IgM,IgG,IgAorIgE,notIgD)κ:λ约2:1，当κ:λ>8-10:1或λ:κ>3:1提示轻链限制表达编辑ppt次级滤泡生发中心、套区和边缘区免疫标记：（1）生发中心(GerminalCenter,GC)：

1）中心细胞和中心母细胞：CD10+,BCL-6+,Bcl-2-,

2）滤泡树突细胞(Folliculardendriticcells,FDC)

:CD21+,CD23+,CD35+3）T细胞：CD4+,

CD10+,CXCL13/PD-1+,少量CD57+4）可染体巨噬细胞：CD68+,lysozyme+,CD11c+（2）套区:处女型B细胞和记忆细胞,表达IgM,IgD

（3）边缘区：无特殊标记，绝大多数次级滤泡难以鉴别，腹腔淋巴结易识别。编辑pptCD20编辑pptCD79a编辑ppt编辑ppt编辑ppt编辑pptCD21编辑pptCD10Bcl-6编辑ppt反应性滤泡增生的特点对T细胞依赖性抗原的反应滤泡增多，大小、形态不等，可弥漫分布，主要位于皮质区，无背靠背现象。典型生发中心具有极性(Polarity):明区和暗区，套区明显，扁桃体组织更易识别；星天现象，中心细胞和中心母细胞混杂，免疫染色：生发中心细胞BCL-2-，ki-67指数较高。滤泡间区一般无B细胞弥漫阳性，B细胞CD43和CD5阴性。编辑ppt编辑ppt编辑pptDarkzoneLightzone编辑ppt

BCL-2

Ki67编辑pptMarginalzonecells编辑pptCD20编辑ppt（三）T淋巴细胞分布：副皮质区(paracortex)T淋巴细胞为主(CD4>CD8):主为小淋巴细胞，偶尔出现大细胞(可以是活化的B细胞);含有高内皮静脉(Highendothelialvenules,HEV):血液淋巴细胞入淋巴结的通道;含有指状树突细胞(interdigitatingdendriticcells,IDC):S100+的专职抗原递呈细胞；编辑ppt编辑ppt编辑pptProthymocyteSubcapsularthymocyteCorticalthymocyteMedullarythymocytePeripheralTcellPrecursorcellsMatureTcellsCytoplasmicSurfaceCD4CD8CD1CD4,CD8CD3CD2CD7TdT编辑ppt编辑pptIDC编辑pptCD3编辑ppt副皮质区增生常见病因:病毒感染,接种疫苗，药物，自身免疫疾病,皮病性淋巴结炎，肿瘤转移等形态特征：副皮质扩大，可形成结节；HEV增生；混合小淋巴细胞和转化淋巴细胞，浆细胞，树突细胞，嗜酸性粒细胞等；免疫染色：主为T细胞，或T/B细胞混合,一般无B细胞为主弥漫分布。编辑ppt编辑ppt编辑pptImmunoblastInfectiousmononucleosis编辑pptCD20CD3

LargecellscomprisemixtureofBandTcells-Reactiveprocess编辑pptCD30+cellsarescatteredandstainingisweakormoderate,whichindicatestheheterogeneity

ofthelargecellpopulation.CD30stainingisdiffuselystrongpositive,whichindicatesthemonocloneentity.

编辑pptKappaLambdaLargeBcellsarepolytypic编辑pptT细胞为主时支持良性的依据（谨慎！！！）：（1）结构不同程度保留：淋巴窦和皮质淋巴滤泡；（2）大细胞群体分布不规则或呈地图样，不形成膨胀性团块；（3）无浸润现象：上皮,脂肪,血管,神经,纤维间质中单行排列；（4）细胞无明显异型：<2.5倍小淋巴细胞；常无透明胞质；核形态较规则，圆形或椭圆形，轻度扭曲或折叠；（5）免疫染色:常为CD4+为主,少为CD8+为主(AIDS,骨髓移植等)；一般无CD4-CD8-或CD4+CD8+表型,PanT+（无缺失）；

少见ALK,TdT,CD56,γδTcellreceptor等表达编辑ppt

斑驳状Autoimmunelymphoproliferativesyndrome，ALPS斑驳状编辑pptReactivelargelymphoidcell(immunoblast)SmalllymphocytesNuclearsizeusuallydoesnotexceed2.5timesthatofsmalllymphocyteNucleususuallyroundorovoid,anddoesnotshowirregularfoldings编辑ppt（四）髓索（medullarycord）富含浆细胞可以清晰或不清晰反应性B细胞的扩张延伸，如反应性淋巴滤泡增生，浆细胞型Castlemandisease。编辑ppt编辑ppt编辑ppt（五）淋巴窦（sinus）输入淋巴管被膜下窦间窦髓窦输出淋巴管；被覆窦内皮(D2-40),含组织细胞和淋巴样细胞；腹腔内淋巴结窦常扩张，较明显；深处窦的存在常提示良性病变。编辑ppt编辑ppt编辑ppt编辑ppt编辑ppt（六）纤维结缔组织网

Connectivetissueframework纤维被膜,延伸至结内的梁索(可见梁索周窦)；正常时可能不清晰；反应性病变常清晰，常有淋巴结周围炎。编辑ppt编辑ppt编辑ppt第三节淋巴瘤分类

WHOClassification2008

非霍奇金淋巴瘤

non-Hodgkinlymphomas

霍奇金淋巴瘤

Hodgkinlymphomas编辑ppt非霍奇金淋巴瘤分类

WHOClassificationof

non-Hodgkinlymphomas(2001)Precursorlymphoblasticlymphoma/leukemia(BorTcelltype)MatureB-cellneoplasmsMatureT-cellandNK-celllymphomas编辑ppt成熟B细胞肿瘤

MatureB-cellneoplasms(WHO)B-cellCLL/SLLB-cellprolymphocyticleukemiaLymphoplasmacyticlymphomaMantlecelllymphomaFollicularlymphomaExtranodalmarginalzoneB-celllymphomaofMALTtypeNodalmarginalzoneB-celllymphomaSplenicmarginalzoneBcelllymphomaHairycellleukemiaDiffuselargeB-celllymphoma(includingmediastinallargeB-celllymphoma)BurkittlymphomaPlasmacytoma,myeloma编辑ppt成熟T细胞或NK细胞肿瘤

MatureT-cell&NK-cellneoplasms(WHO)T-cellprolymphocyticleukemiaT-celllargegranularlymphocyticleukemiaAggressiveNKcellleukemiaExtranodalNK/Tcelllymphoma,nasal-typeMycosisfungoides,SezarysyndromeAngioimmunoblasticTcelllymphomaPeripheralT-celllymphoma,unspecifiedAdultT-cellleukemia/lymphomaAnaplasticlargecelllymphoma(Tornullcell),primarysystemictypePrimarycutaneousanaplasticlargecelllymphomaSubcutaneouspanniculitis-likeT-celllymphomaEnteropathy-typeT-celllymphomaHepatosplenicT-celllymphoma编辑ppt非霍奇金淋巴瘤临床生物学行为分类Indolentlymphoma(“Good”)Aggressivelymphoma

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

Indolentlymphomas:

smallcelllymphoma

FollicularlymphomaB-cellCLL/smalllymphocyticlymphomaLymphoplasmacyticlymphomaSplenicandnodalmarginalzonelymphoma编辑ppt惰性淋巴瘤临床特征UsuallyolderadultsSlow-growing;occasionallywaxingandwaningUsuallyhighstage(III/IV)atpresentation;blood&marrowinvolvementcommonTakesmanyyearstokillpatientifuntreatedLowproliferativefraction:oftenincurable.Despitetreatment(diseasecontrol),repeatedrelapseistheruleTreatmentdoesnotaltertheeventualoutcome,thusobservationwithouttreatmentisanoption编辑pptSurvivalcurveofindolentlymphomas%Survival100%0%Years010编辑ppt侵袭性淋巴瘤

Aggressivelymphomas:

largecelllymphoma

DiffuselargeB-celllymphomaMantlecelllymphoma*

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

%Survival100%0%Years010AggressiveNHLIndolentNHLParadoxicallybetteroutcomeinthelongrun编辑ppt高度侵袭性淋巴瘤

Highlyaggressivelymphomas:

Medium-sizedcelllymphoma

Lymphoblasticlymphoma

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

LocalizedindolentlymphomasExtranodalmarginalzoneB-celllymphomaofmucosa-associatedlymphoidtissue

（MALT）

typePrimarycutaneousanaplasticlargecelllymphoma编辑pptLocalizedindolentlymphomasIndolenttumorCanoccurinanyageOftenlocalizedatpresentationManypatientscanapparentlybecured(thereisasuggestionofplateauinsurvivalcurve),althoughlaterelapsesarepossibleCanundergoregressionspontaneousforprimarycutaneousALCLwithanti-HelicobactertherapyforgastricextranodalMZL编辑ppt各型淋巴瘤的临床病理特征编辑ppt淋巴母细胞淋巴瘤RapidlygrowingtumorLymphoma:T>>BLeukemia(ALL):B>>TUntreated:rapidlyfatalAggressivetherapy:goodsurvivalCannotpredictlineagefrommorphologyDefiningmarker:TdT+，CD99编辑ppt编辑ppt编辑pptCD20CD79aB-LBL编辑pptT-LBL编辑ppt滤泡性淋巴瘤

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

t(14;18)(q32;q21)

bcl-2generearrangement

(duetoBCL2/IGHtranslocation)编辑pptFollicularlymphomavs

reactivefollicularhyperplasiaMajorcriterionCloselypackedfolliclesthroughout,withscantyinterfolliculartissues1majorcriterionor3minorcriteria.Butconfirmbyancillarytestsifuncertain编辑ppt滤泡性淋巴瘤主要诊断标准示意图

Majorcriterionfulfilledin80%ofcasesoffollicularlymphomaJustgoontohighmagnificationtoconfirmfolliclecentercellmorphology编辑ppt编辑ppt编辑pptCentrocytesoftenhavetriangularshapednuclei编辑ppt滤泡性淋巴瘤的次要诊断标准Intheremaining20%ofcases(non-crowdedfollicles),acombinationofminorcriteria(3)

havetobeconsidered,andsupplementedbyancillarystudiesasrequired编辑pptMinorcriteria

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

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

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

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

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

t(11;14)(q13;q32)

orcryptictranslocation编辑pptCyclinD1编辑ppt

粘膜相关淋巴组织

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

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

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

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

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

Tcelllymphoma编辑pptPeripheralTcelllymphoma

外周T细胞淋巴瘤PredominantlynodalAngioimmunoblasticT-celllymphomaAnaplasticlargecelllymphoma,T/null-cellPeripheralT-celllymphoma,unspecified编辑pptANGIOIMMUNOBLASTIC

T-CELLLYMPHOMA

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

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

immunophenotypeCD2+.sCD3-,cCD3e+,CD56+

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

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

MorphologiccluesforrecognitionPredominantparacorticalinvolvementProminenthighendothelialvenulesSpectrumofcellssizesandshapesIrregularnuclearcontoursClearcellsManyadmixedepithelioidhistiocytesandeosinophilsButimmunostainingessentialforconfirmingTlineage编辑ppt编辑ppt编辑pptANAPLASTICLARGECELLLYMPHOMA,primarysystemicform

间变性大细胞淋巴瘤，原发系统性TendstooccurinchildrenandyoungadultsNodalorextranodalpresentation(whichmayincludeskin,softtissue,bone,G.I.tract)Immunophenotype:CD30+;EMAusually+T-lineagemarkers+/-(Tornullcell)AproportionofcasesexpressALKduetotranslocationinvolvingALKgene编辑pptHallmarkcells编辑pptALKtranslocation(orexpression)hashelpedtodefineahomogeneoussubgroupofALCLThiscanbeeasilydetectedbyimmunostainingforALK--permittingre-definitionofthemorphologicspectrumofALCL:ClassicaltypeMonomorphicSmallcellLymphohistiocyticHypocellular编辑ppt编辑pptCD30编辑ppt

Overallsurvival

PrimarysystemicformofALCL

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

霍奇金淋巴瘤

HODGKINLYMPHOMA编辑pptLymphocytepredominanceMixedcellularityNodularsclerosisLymphocytedepletionNodularlymphocytepredominantHodgkinlymphoma

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