内科学-淋巴瘤-邹善华PPT课件

.,1,LymphomaZhongshanHospital,FudanUniversityZouShanhua,.,2,Definition：AgroupofneoplasticdiseasesoflymphoidtissuesClassification：HodgkinsDisease,HD(HodgkinsLymphoma)NonHodgkinsLymphoma,NHL),GeneralConsiderations,.,3,ClinicalFeatures：Painless,progressivelymphadenectasisFever,weightloss,nightsweat；cachexiaHepatosplenomegalyEpidemiology：Incidence：incities/inthecountry;male/female;ageHD811；NHL90%,GeneralConsiderations,.,4,Virulogy:EBvirusBurkittlymphoma,HDHumanT-cellLymphotropicVirustype1,HTLV-1AdultT-celllymphoma/leukemia,ATLHTLV-2MycosisFungoidesHelicobacterPyloriMALTlymphomaKaposisarcoma-associatedherpesvirusprimarybodycavitylymphoma,EtiologyandPathogenesis,.,5,ImmuneStatesofHost：Immunodeficiency/immunosuppressionMechanism：Antigenicstimulationlymphadenosis(modulation)infiniteproliferationlymphoma,EtiologyandPathogenesis,.,6,ClassificationofLymphoma,HD：Reed-Sternberg(R-S)Cell,.,7,ClassificationofLymphoma,RyeClassification，1966LymphocytepredominanceNodularsclerosisMixedcellularityLymphocytedepletion,.,8,ClassificationofLymphoma,WHOClassification,2001NodularlymphocytepredominanceHodgkinslymphomaClassicalHodgkinslymphomaLymphocyte-richclassicalHodgkinslymphomaNodularsclerosisHodgkinslymphomaMixedcellularityHodgkinslymphomaLymphocytedepletionHodgkinslymphoma,.,9,ClassificationofLymphoma,NHL：,.,10,ClassificationofLymphoma,NHL：IWF，1982REAL，1994WHO，2001,.,11,WorkingFormulation,Low-gradelymphomaA.smalllymphocytic,consistentwithchroniclymphocyticleukemiaplasmacytoidB.follicular,predominantlysmallcleavedcelldiffuseareassclerosisC.follicular,mixedsmallcleavedandlargecelldiffuseareassclerosis,.,12,WorkingFormulation,Intermediate-gradlymphomaD.follicular,predominantlylargecelldiffuseareassclerosisE.diffuse,smallcleavedcellsclerosisF.diffuse,mixedsmallandlargecellsclerosisepithelioidcellcomponentG.diffuse,largecellcleavedcellnoncleavedcellsclerosis,.,13,WorkingFormulation,High-gradelymphomaH.mmunoblastic,largecellplasmacytoidclearcellpolymorphousepithelioidcellcomponentI.lymphoblastic,convolutedornonconvolutedcellJ.smallnoncleavedcell,Burkittsornon-Burkitts,.,14,WorkingFormulation,Miscellaneouscompositemycosisfungoideshistiocyticextramedullaryplasmacytomaunclassifiableother,.,15,REALClassification,B-cellneoplasmsI.PrecursorB-cellneoplasm:precursorB-lymphoblasticleukemia/lymphomaII.PeripheralB-cellneoplasmsA.B-cellchroniclymphocyticleukemia/prolymphocyticleukemia/smalllymphocyticlymphomaB.Lymphoplasmacytoidlymphoma/immunocytomaC.MantlecelllymphomaD.Folliclecentercelllymphoma,follicular1.Provisionalcytologicgrades:Ismallcell,IImixedsmallandlargecell,IIIlargecell2.Provisionalsubtype:diffuse,predominantlysmallcelltype,.,16,REALClassification,E.MarginalzoneB-celllymphoma1.Extranodal(MALT-type+/-monocytoidBcells)2.Provisionalsubtype:nodal(+/-monocytoidBcells)F.Provisionalentity:splenicmarginalzonelymphoma(+/-villouslymphocytes)G.HairycellleukemiaH.Plasmacytoma/plasmacellmyelomaI.DiffuselargeB-celllymphoma1.Subtype:primarymediastinal(thymic)B-celllymphomaJ.BurkittslymphomaK.Provisionalentity:high-gradeB-celllymphoma,Burkitt-like,.,17,REALClassification,T-cellandputativeNK-cellneoplasmsI.PrecursorT-cellneoplasm:precursorT-lymphoblasticlymphoma/leukemiaII.PeripheralT-cellandNK-cellneoplasmsA.T-cellchroniclymphocyticleukemia/prolymphocyticleukemiaB.Largegranularlymphocyteleukemia1.T-celltype2.NK-celltypeC.Mycosisfungoides/Sezarysyndrome,.,18,REALClassification,D.PeripheralT-celllymphomas,unspecified1.Provisionalcytologiccategories:medium-sizedcell,mixedmediumandlargecell,largecell,lymphoepithelioidcell2.Provisionalsubtype:hepatosplenicgamma/deltaT-celllymphoma3.Provisionalsubtype:subcutaneouspanniculiticT-celllymphomaE.AngioimmunoblasticTcelllymphomaF.Angiocentriclymphoma,.,19,REALClassification,G.IntestinalT-celllymphoma(+/-enteropathyassociated)H.AdultT-celllymphoma/leukemiaI.Anaplasticlargecelllymphoma1.CD30+-celltype2.T-celltype3.Null-celltypesJ.Provisionalentity:anaplasticlargecelllymphoma,Hodgkins-like,.,20,WHOClassification,2001,B-cellneoplasmsI.PrecursorB-cellneoplasm:a.PrecursorB-lymphoblasticleukemia/lymphomaII.Mature(peripheral)B-cellneoplasmsaB-cellchroniclymphocyticleukemia/smalllymphocyticlymphomabB-cellprolymphocyticleukemiacLymphoplasmacyticlymphomadSpenicmarginalzoneB-celllymphoma(+/-villouslymphocytes)eNodalmarginalzonelymphoma(+/-monocytoidB-cells)fExtranodalmarginalzoneB-celllymphomaofmucosa-associatedlymphoidtissue(MALT)type,.,21,WHOClassification,2001,gHairycellleuekmiahPlasmacellmyeloma/plasmacytomaiFollicularlymphoma,folliclecenterjMantlecelllymphomakDiffuselargecellB-celllymphomaMediastinallargeB-celllymphomaIntravascularlargeB-celllymphomaPrimaryeffusionlymphomalBurkittslymphoma/BurkittscellleukemiaB-cellproliferationsofuncertainmalignantpotentialLymphomatoidgranulomatosisPost-transplantlymphoproliferativedisorder,.,22,WHOClassification,2001,T-CellandNaturalKillerCellNeoplasmsI.PrecursorTcellneoplasm:a.PrecursorT-lymphoblasticlymphoma/leukemiab.BlasticNKlymphoma,.,23,WHOClassification,2001,II.Mature(peripheral)TcellandNK-cellneoplasmsaTcellprolymphocyticleukemiabT-cellgranularlymphocyticleukemiacAggressiveNKCellleukemiadAdultTcelllymphoma/leukemia(HTLV1+)eExtranodalNK/T-celllymphoma,nasaltypefEnteropathy-typeT-celllymphomagHepatosplenicgamma-deltaT-celllymphomahSubcutaneouspanniculitis-likeT-celllymphomaiMycosisfungoides/Sezaryssyndrome,.,24,WHOClassification,2001,jPrimaryCutaneousAnaplasticlargecelllymphomaT/nullcellkPeripheralTcelllymphoma,unspecifiedlAngioimmunoblasticTcelllymphomamPrimarySystemicAnaplasticlargecelllymphoma,T/nullcellT-cellproliferationofuncertainmalignantpotentialLymphomatoidpapulosis,.,25,ClinicalFeatures,.,26,.,27,LabandOtherFindings,PeripheralBloodandBoneMarrowHD：Eosinophilia；RScellNHL：Lymphocytosis;ComplicatedleukemiaLDH，2-MG,Coombstest，globulinRadiologicstudiesChestX-rayCT,MRIRadioactivenuclidePET-CTB-ultrasoundBiopsyImmunophenotypeCytogeneticandMolecularStudies,.,28,DiagnosisPainless,progressivelymphadenectasisBiopsySkinLesionBiopsyBoneMarrowAspirationandBiopsyBiopsyofOtherSites,DiagnosisandDifferentialDiagnosis,.,29,Stagingoflymphoma,StageDefinitionInvolvementofasinglelymphnoderegionorofasingleextranodalorganorsite(E)Involvementoftwoormorenoderegionsonthesamesideofthediaphragm,orlocalizedinvolvementofanextranodalsiteororgan(E)andoneormorelymphnoderegionsonthesamesideofthediaphragmInvolvementoflymphnoderegionsonbothsidesofthediaphragmwhichmayalsobeaccompaniedbylocalizedinvolvementofanextranodalorgansorsite(E)orspleen(s)orboth(sE)DiffuseordisseminatedinvolvementofoneormoredistantextranodalorganswithorwithoutassociatedlymphnodeinvolvementBsymptomsFever38,nightsweats,and/orweightloss10%ofbodyinthe6months,.,30,Stagingoflymphoma,.,31,DifferentialDiagnosisLymphadenectasis：ChroniclymphadenitisTuberculouslymphadenitisTumournodemetastasesFever：Septicaemia,Tuberculosis,RheumaticdiseaseExtranodalLymphoma:Othermalignanttumer,DiagnosisandDifferentialDiagnosis,.,32,RadiationTherapy60Co,acceleratorlinearDosage:3040GY，34WMethods：