淋巴组织肿瘤

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淋巴组织肿瘤DrJohnK.C.CHAN陈国璋淋巴组织肿瘤4thEditionPublished20/9/2008淋巴组织肿瘤第一节概述一、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%）

淋巴组织肿瘤(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淋巴组织肿瘤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。淋巴组织肿瘤二、淋巴结的正常免疫结构（一）淋巴结结构:皮质,髓质,窦,被膜和纤维小梁淋巴组织肿瘤（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。淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤PrepreBcellPreBcellImmatureBcellMatureBcellActivatedBcellPlasmacellPrecursorcells;antigennon-responsiveVirginBcells;antigenresponsiveGerminalcenter&postGCcells;antigenencounteredTdTCD20,CD22CD19,

PAX5CD79aCD10Bcl-6CD138CytoplasmicCD22淋巴组织肿瘤ImmunoarchitectureofnormallymphnodeBcells(CD20+,CD10+,BCL-6+)Tcells(CD3+++);onlysmallnumbersofCD20+cells淋巴组织肿瘤淋巴组织肿瘤

1、初级滤泡：处女型B细胞和成熟小淋巴细胞；

淋巴组织肿瘤CD202、次级滤泡：由生发中心、套区和边缘区构成。淋巴组织肿瘤Organizationofsecondarylymphoidfollicle

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提示轻链限制表达淋巴组织肿瘤次级滤泡生发中心、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。淋巴组织肿瘤CD20淋巴组织肿瘤CD79a淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤CD21淋巴组织肿瘤CD10Bcl-6淋巴组织肿瘤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。淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤DarkzoneLightzone淋巴组织肿瘤

BCL-2

Ki67淋巴组织肿瘤Marginalzonecells淋巴组织肿瘤CD20淋巴组织肿瘤（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；淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤ProthymocyteSubcapsularthymocyteCorticalthymocyteMedullarythymocytePeripheralTcellPrecursorcellsMatureTcellsCytoplasmicSurfaceCD4CD8CD1CD4,CD8CD3CD2CD7TdT淋巴组织肿瘤淋巴组织肿瘤IDC淋巴组织肿瘤CD3淋巴组织肿瘤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。淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤ImmunoblastInfectiousmononucleosis淋巴组织肿瘤CD20CD3

LargecellscomprisemixtureofBandTcells-Reactiveprocess淋巴组织肿瘤CD30+cellsarescatteredandstainingisweakormoderate,whichindicatestheheterogeneity

ofthelargecellpopulation.CD30stainingisdiffuselystrongpositive,whichindicatesthemonocloneentity.

淋巴组织肿瘤KappaLambdaLargeBcellsarepolytypic淋巴组织肿瘤T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等表达淋巴组织肿瘤

斑驳状Autoimmunelymphoproliferativesyndrome，ALPS斑驳状淋巴组织肿瘤Reactivelargelymphoidcell(immunoblast)SmalllymphocytesNuclearsizeusuallydoesnotexceed2.5timesthatofsmalllymphocyteNucleususuallyroundorovoid,anddoesnotshowirregularfoldings淋巴组织肿瘤（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。淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤（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。淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤（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。淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤第三节淋巴瘤分类

WHOClassification2008

非霍奇金淋巴瘤

non-Hodgkinlymphomas

霍奇金淋巴瘤

Hodgkinlymphomas淋巴组织肿瘤非霍奇金淋巴瘤分类

WHOClassificationof

non-Hodgkinlymphomas(2001)Precursorlymphoblasticlymphoma/leukemia(BorTcelltype)MatureB-cellneoplasmsMatureT-cellandNK-celllymphomas淋巴组织肿瘤成熟B细胞肿瘤

MatureB-cellneoplasms(WHO)B-cellCLL/SLLB-cellprolymphocyticleukemiaLymphoplasmacyticlymphomaMantlecelllymphomaFollicularlymphomaExtranodalmarginalzoneB-celllymphomaofMALTtypeNodalmarginalzoneB-celllymphomaSplenicmarginalzoneBcelllymphomaHairycellleukemiaDiffuselargeB-celllymphoma(includingmediastinallargeB-celllymphoma)BurkittlymphomaPlasmacytoma,myeloma淋巴组织肿瘤成熟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淋巴组织肿瘤非霍奇金淋巴瘤临床生物学行为分类Indolentlymphoma(“Good”)Aggressivelymphoma

(“Bad”)Highlyaggressivelymphoma(“Ugly”)Unusuallocalizedindolentlymphoma淋巴组织肿瘤惰性淋巴瘤

Indolentlymphomas:

smallcelllymphoma

FollicularlymphomaB-cellCLL/smalllymphocyticlymphomaLymphoplasmacyticlymphomaSplenicandnodalmarginalzonelymphoma淋巴组织肿瘤惰性淋巴瘤临床特征UsuallyolderadultsSlow-growing;occasionallywaxingandwaningUsuallyhighstage(III/IV)atpresentation;blood&marrowinvolvementcommonTakesmanyyearstokillpatientifuntreatedLowproliferativefraction:oftenincurable.Despitetreatment(diseasecontrol),repeatedrelapseistheruleTreatmentdoesnotaltertheeventualoutcome,thusobservationwithouttreatmentisanoption淋巴组织肿瘤Survivalcurveofindolentlymphomas%Survival100%0%Years010淋巴组织肿瘤侵袭性淋巴瘤

Aggressivelymphomas:

largecelllymphoma

DiffuselargeB-celllymphomaMantlecelllymphoma*

AllperipheralTcelllymphomas(includingALCL&NKcelllymphomasexcept:Mycosisfungoides)PrimarycutaneousanaplasticlargecelllymphomaT-cellLGLleukemia淋巴组织肿瘤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淋巴组织肿瘤Survivalcurveofaggressivelymphomas%Survival100%0%Years010TreatedUntreated淋巴组织肿瘤Comparisonofsurvivalcurves

%Survival100%0%Years010AggressiveNHLIndolentNHLParadoxicallybetteroutcomeinthelongrun淋巴组织肿瘤高度侵袭性淋巴瘤

Highlyaggressivelymphomas:

Medium-sizedcelllymphoma

Lymphoblasticlymphoma

Burkittlymphoma淋巴组织肿瘤HighlyaggressivelymphomasChildren,youngadultsVeryrapidlygrowing:oncologicemergencyOftenhighstage;marrowinvolvementcommon;CNSinvolvementmayoccurUntreated,willkillinweekstomonthsVeryhighproliferativefraction:highlyresponsivetotreatment.Standardchemotherapy:nogood;veryaggressivechemotherapy:manycured淋巴组织肿瘤Survivalcurveofhighlyaggressivelymphomas%Survival100%0%Years010TreatedUntreated淋巴组织肿瘤局限性惰性淋巴瘤

LocalizedindolentlymphomasExtranodalmarginalzoneB-celllymphomaofmucosa-associatedlymphoidtissue

（MALT）

typePrimarycutaneousanaplasticlargecelllymphoma淋巴组织肿瘤LocalizedindolentlymphomasIndolenttumorCanoccurinanyageOftenlocalizedatpresentationManypatientscanapparentlybecured(thereisasuggestionofplateauinsurvivalcurve),althoughlaterelapsesarepossibleCanundergoregressionspontaneousforprimarycutaneousALCLwithanti-HelicobactertherapyforgastricextranodalMZL淋巴组织肿瘤各型淋巴瘤的临床病理特征淋巴组织肿瘤淋巴母细胞淋巴瘤RapidlygrowingtumorLymphoma:T>>BLeukemia(ALL):B>>TUntreated:rapidlyfatalAggressivetherapy:goodsurvivalCannotpredictlineagefrommorphologyDefiningmarker:TdT+，CD99淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤CD20CD79aB-LBL淋巴组织肿瘤T-LBL淋巴组织肿瘤滤泡性淋巴瘤

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

t(14;18)(q32;q21)

bcl-2generearrangement

(duetoBCL2/IGHtranslocation)淋巴组织肿瘤Follicularlymphomavs

reactivefollicularhyperplasiaMajorcriterionCloselypackedfolliclesthroughout,withscantyinterfolliculartissues1majorcriterionor3minorcriteria.Butconfirmbyancillarytestsifuncertain淋巴组织肿瘤滤泡性淋巴瘤主要诊断标准示意图

Majorcriterionfulfilledin80%ofcasesoffollicularlymphomaJustgoontohighmagnificationtoconfirmfolliclecentercellmorphology淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤Centrocytesoftenhavetriangularshapednuclei淋巴组织肿瘤滤泡性淋巴瘤的次要诊断标准Intheremaining20%ofcases(non-crowdedfollicles),acombinationofminorcriteria(3)

havetobeconsidered,andsupplementedbyancillarystudiesasrequired淋巴组织肿瘤Minorcriteria

Notingible-bodymacrophagesCellularmonotony:centrocytepredominanceNocellularpolarisationAbsentorincompletemantlesFolliclesinperinodaltissuesDysplasticfollicularcentercells,e.g.signetringcells,frequentcytoplasmicglobulesAtypicalcellsininterfolliculartissuesInvasionofwallsofbloodvessels淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤FollicularlymphomavsRFH:ancillarytechniquesImmunostainingforbcl-2MostusefulimmunostainNormal:BothTandBcellspositiveexceptfolliclecentercellsLookforpositivestainingofcellsinfolliclecenters:positivestainingsupportsdiagnosisoflymphoma淋巴组织肿瘤Reactivefollicles淋巴组织肿瘤Follicularlymphoma(Bcl-2immunostaining)淋巴组织肿瘤Commonmisconceptionsinhematopathology"Negativebcl-2stainrulesoutfollicularlymphoma"Thetruth:Itdoesnot.20-25%offollicularlymphomacasesarenegative.Pursuewithotherteststoconfirmorrefuteadiagnosisoffollicularlymphoma淋巴组织肿瘤Bcl-2stainingin

follicularlymphomaGrade(Cytologiccomposition)Bcl-2immunoreactivityI(predominantlysmallcells)100%II(mixedsmallandlargecells)85%III(predominantlylargecells)75%淋巴组织肿瘤淋巴组织肿瘤Bcl-2淋巴组织肿瘤LookforevidenceinterfollicularinvasionDenselypackedCD20+orCD79+B-cellsbetweenfolliclesisindicativeofinterfollicularinvasionPresenceofasignificantnumberofCD10+lymphoidcells(notneutrophils,whicharealsoCD10+)betweenfolliclesalsoindicatesinterfollicularinvasionLookforvascularinvasion

ManyCD10+cellsinthewallsofperinodalbloodvessels淋巴组织肿瘤CD20淋巴组织肿瘤CD10淋巴组织肿瘤淋巴组织肿瘤LookformonoclonalityorlowproliferationindexImmunostainforIgtolookforlightchainrestrictionDemonstratedbyKi67,alowKi67indexfavorsdiagnosisoffollicularlymphomaoverRFH(meanindex15.6%vs64.9%);ahighKi67indexisinconclusiveMolecularanalysisIggenerearrangementbySouthernblotorPCR(NotethatPCRmayhavefalsenegativeresults)DetectionofBCL2generearrangementbySouthernblot,PCRorFISH淋巴组织肿瘤KappaLambda淋巴组织肿瘤FL1:Ki67RFH:Ki67淋巴组织肿瘤FL3:Ki67淋巴组织肿瘤套细胞淋巴瘤

Mantlecelllymphoma:morphologyDiffuse,vaguelynodular,ormantlezonepatternBroadcytologicspectrumClassictype:smalllymphoidcellswithirregulartoroundnucleiBlastoidvariant(~lymphoblasts,butTdT-)PleomorphicvariantOftenwithsolitaryepithelioidhistiocytesInterspersed‘nakednuclei’(folliculardendriticcells)Genetics:Iggenerearrangementt(11,14)(q13;q32)淋巴组织肿瘤GrowthpatternofMCL淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤Roundnuclei淋巴组织肿瘤Mantlecelllymphoma:

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

t(11;14)(q13;q32)

orcryptictranslocation淋巴组织肿瘤CyclinD1淋巴组织肿瘤

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淋巴组织肿瘤淋巴组织肿瘤LOWGRADEBCELLMALTLYMPHOMA(extranodalmarginalzoneB-celllymphoma)Usuallylocalizedatpresentation(66-79%)RecurrenceatotherMALTsites(“homing”)Manycanbetreatedbylocoregionaltherapyoreradicationofantigen-stimulationsourceCompleteremissionrate=72%5-yearoverallsurvival=74%(Gastric:90%)淋巴组织肿瘤Histologicfeaturesoflowgrade

MALTlymphomaSmall/medium-sizedcells(lymphocytes,centrocyte-like,monocytoidBcells)Patterns:diffuse,interfollicular,marginalzoneLymphoepitheliallesionsPlasmacellsarecommonReactivelymphoidfollicles+/-colonization淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤LowgradeMALTlymphoma:ImmunophenotypeAneoplasmofpost-germinalcenterBcellsPanB+,CD10-,CD5-,CyclinD1-,CD23-Keyfeatures:siteofinvolvementlymphoepitheliallesionexclusionofotherlowgradeB-celllymphomas淋巴组织肿瘤AfterHelicobactereradicationtherapy淋巴组织肿瘤弥漫性大B细胞淋巴瘤

DIFFUSELARGEBCELLLYMPHOMA（DLBCL）CommonestnodalB-celllymphomaAheterogeneousentityFinalcommonpathwayforhighgradetransformationoflowgradeB-celllymphomas淋巴组织肿瘤Centroblastic/Immunoblastic淋巴组织肿瘤淋巴组织肿瘤MultilobatedPleomorphic淋巴组织肿瘤BURKITTLYMPHOMAThreedifferentforms:Endemic(EquatorialAfrica;strongassociationwithEBV>90%;commonlyaffectingjaws,gonads,kidneys)Sporadic(EBVassociationonly~15%;commonlyinvolvingterminalileumandWaldeyer’sring)AIDS-related(EBVassociation~30%;commonlyinvolvinglymphonodeandbonemarrow)AfricancasescanalsobeAIDS-related!淋巴组织肿瘤Burkittlymphomat(8;14)(q24;q32)orvariants,withtranslocationbetweenc-mycandIggeneMostlyadiseaseofyoungpatientsHighlyaggressive,butpotentiallycurablebychemotherapy淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤Burkittlymphoma:ImmunogeneticsProbablycorrespondstoIgM+blastsingerminalcenterPan-B+,CD10+,Bcl-6+,Bcl-2-Keyfeatures:C-MYCtranslocationmedium-sizedcellshighKi67index：～100%germinalcentercellphenotype淋巴组织肿瘤CD20CD10淋巴组织肿瘤Ki67淋巴组织肿瘤外周T和NK细胞淋巴瘤

PERIPHERALTANDNKCELLLYMPHOMASUncommon;<10%ofallnon-Hodgkin’slymphomasinCaucasians,but15-20%inChineseLessfavorableoutcomecomparedwithaggressiveBcelllymphomas淋巴组织肿瘤TandNKcelllymphomas:

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

Tcelllymphoma淋巴组织肿瘤PeripheralTcelllymphoma

外周T细胞淋巴瘤PredominantlynodalAngioimmunoblasticT-celllymphomaAnaplasticlargecelllymphoma,T/null-cellPeripheralT-celllymphoma,unspecified淋巴组织肿瘤ANGIOIMMUNOBLASTIC

T-CELLLYMPHOMA

血管免疫母细胞性T细胞淋巴瘤CommonesttypeofnodalTcelllymphomaFever,skinrash,systemicsymptomsGeneralizedlymphnodesIncreasedserumIg(polyclonal);CoombspositivehemolyticanemiaAlthoughsomerespondtosteroid,prognosisisunfavorable;succumbingtoinfectioncomplications淋巴组织肿瘤AneoplasmoffollicularcenterThelpercellsPanT+,usuallyCD4+(butalsomanyCD8+cells),CD10+,CXCL13+,PD-1Keyfeatures:ArborizingvenulesLymphoidcellsoftenwithroundnuclei;clearcellscommonirregularmeshworksoffolliculardendriticcellaroundvenulesCD10/CXCL13+TcellsoutsidefollicleIsolatedEBV+cells(mostlyBcells)淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤CD3CD21淋巴组织肿瘤CXCL13淋巴组织肿瘤EXTRANODALNK/TCELLLYMPHOMA

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

immunophenotypeCD2+.sCD3-,cCD3e+,CD56+

Cytotoxicgranulesperforin,granzymeB,TIA-1positiveCD4,CD5,CD7,CD8:usuallynegativeKeyfeatures:CD56+,EBER+IfCD56-,requiresEBER+andcytotoxicgranules+fordiagnosis淋巴组织肿瘤SurfaceCD3CytoplasmicCD3εCD56FrozentissueParaffinsection淋巴组织肿瘤PERIPHERALTCELLLYMPHOMAUNSPECIFIED

外周T细胞淋巴瘤，非特殊型AwastebasketcategoryVariableclinicalmanifestationsUsuallyhighstagedisease(nodal/extranodal)MayhaveeosinophiliaAggressive;relapsecommonTheextranodalcasesoftenexpressacytotoxiccellphenotype淋巴组织肿瘤PeripheralT-celllymphoma:

MorphologiccluesforrecognitionPredominantparacorticalinvolvementProminenthighendothelialvenulesSpectrumofcellssizesandshapesIrregularnuclearcontoursClearcellsManyadmixedepithelioidhistiocytesandeosinophilsButimmunostainingessentialforconfirmingTlineage淋巴组织肿瘤淋巴组织肿瘤淋巴组织肿瘤ANAPLASTICLARGECELLLYMPHOMA,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淋巴组织肿瘤Hallmarkcells淋巴组织肿瘤ALKtranslocation(orexpression)hashelpedtodefineahomogeneoussubgroupofALCLThiscanbeeasilydetectedbyimmunostainingforALK--permittingre-definitionofthemorphologicspectrumofALCL:ClassicaltypeMonomorphicSmallcellLymphohistiocyticHypocellular淋巴组织肿瘤淋巴组织肿瘤CD30淋巴组织肿瘤

Overallsurvival

PrimarysystemicformofALCL

0510YearsALCL,ALK+*100%50%ALCL,ALK-淋巴组织肿瘤

霍奇金淋巴瘤

HODGKINLYMPHOMA淋巴组织肿瘤LymphocytepredominanceMixedcellularityNodularsclerosisLymphocytedepletionNodularlymphocytepredominantHodgkinlymphoma

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