內(nèi)科學(xué)淋巴瘤鄒善華

內(nèi)科學(xué)淋巴瘤鄒善華Definition：AgroupofneoplasticdiseasesoflymphoidtissuesClassification：

Hodgkin′sDisease,HD(Hodgkin′sLymphoma)NonHodgkin′sLymphoma,NHL)GeneralConsiderations2021/4/272ClinicalFeatures：

Painless,progressivelymphadenectasisFever,weightloss,nightsweat；cachexiaHepatosplenomegalyEpidemiology：

Incidence：incities/inthecountry;male/female;ageHD8～11％；NHL90%GeneralConsiderations2021/4/273Virulogy:

EBvirus→Burkittlymphoma,HD

HumanT-cellLymphotropicVirustype1,HTLV-1→AdultT-celllymphoma/leukemia,ATL

HTLV-2→MycosisFungoidesHelicobacterPylori→MALTlymphomaKaposisarcoma-associatedherpesvirus→primarybodycavitylymphoma

EtiologyandPathogenesis

2021/4/274ImmuneStatesofHost：

Immunodeficiency/immunosuppressionMechanism：Antigenicstimulation→

lymphadenosis(modulation↓)→infiniteproliferation→lymphomaEtiologyandPathogenesis2021/4/275ClassificationofLymphomaHD：Reed-Sternberg(R-S)Cell

2021/4/276ClassificationofLymphoma

RyeClassification，1966LymphocytepredominanceNodularsclerosisMixedcellularityLymphocytedepletion2021/4/277ClassificationofLymphoma

WHOClassification,2001NodularlymphocytepredominanceHodgkin'slymphomaClassicalHodgkin'slymphomaLymphocyte-richclassicalHodgkin'slymphomaNodularsclerosisHodgkin'slymphomaMixedcellularityHodgkin'slymphomaLymphocytedepletionHodgkin'slymphoma2021/4/278ClassificationofLymphomaNHL：2021/4/279ClassificationofLymphomaNHL：

IWF，1982REAL，1994WHO，20012021/4/2710WorkingFormulation

Low-gradelymphomaA.smalllymphocytic,consistentwithchroniclymphocyticleukemiaplasmacytoidB.follicular,predominantlysmallcleavedcelldiffuseareassclerosisC.

follicular,mixedsmallcleavedandlargecelldiffuseareas

sclerosis2021/4/2711WorkingFormulationIntermediate-gradlymphomaD.

follicular,predominantlylargecelldiffuseareassclerosisE.diffuse,smallcleavedcellsclerosisF.

diffuse,mixedsmallandlargecellsclerosisepithelioidcellcomponentG.

diffuse,largecellcleavedcellnoncleavedcellsclerosis

2021/4/2712WorkingFormulationHigh-gradelymphomaH.mmunoblastic,largecellplasmacytoidclearcellpolymorphousepithelioidcellcomponentI.lymphoblastic,convolutedornonconvolutedcellJ.smallnoncleavedcell,Burkitt'sornon-Burkitt's2021/4/2713WorkingFormulationMiscellaneouscompositemycosisfungoideshistiocyticextramedullaryplasmacytomaunclassifiableother2021/4/2714REALClassification

B-cellneoplasmsI.PrecursorB-cellneoplasm:precursorB-lymphoblasticleukemia/lymphomaII.PeripheralB-cellneoplasms

A.B-cellchroniclymphocyticleukemia/prolymphocyticleukemia/smalllymphocyticlymphomaB.Lymphoplasmacytoidlymphoma/immunocytomaC.MantlecelllymphomaD.Folliclecentercelllymphoma,follicular1.Provisionalcytologicgrades:Ismallcell,IImixedsmallandlargecell,IIIlargecell2.Provisionalsubtype:diffuse,predominantlysmallcelltype

2021/4/2715REALClassificationE.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.Burkitt’slymphomaK.Provisionalentity:high-gradeB-celllymphoma,Burkitt-like

2021/4/2716REALClassificationT-cellandputativeNK-cellneoplasmsI.PrecursorT-cellneoplasm:precursorT-lymphoblasticlymphoma/leukemiaII.PeripheralT-cellandNK-cellneoplasmsA.T-cellchroniclymphocyticleukemia/prolymphocyticleukemiaB.Largegranularlymphocyteleukemia1.T-celltype2.NK-celltypeC.Mycosisfungoides/Sezary’syndrome2021/4/2717REALClassification

D.PeripheralT-celllymphomas,unspecified1.Provisionalcytologiccategories:medium-sizedcell,mixedmediumandlargecell,largecell,lymphoepithelioidcell2.Provisionalsubtype:hepatosplenicgamma/deltaT-celllymphoma3.Provisionalsubtype:subcutaneouspanniculiticT-celllymphomaE.AngioimmunoblasticTcelllymphomaF.Angiocentriclymphoma2021/4/2718REALClassification

G.IntestinalT-celllymphoma(+/-enteropathyassociated)H.AdultT-celllymphoma/leukemiaI.Anaplasticlargecelllymphoma1.CD30+-celltype2.T-celltype3.Null-celltypesJ.Provisionalentity:anaplasticlargecelllymphoma,Hodgkin's-like

2021/4/2719WHOClassification,2001B-cellneoplasmsI.PrecursorB-cellneoplasm:

a.PrecursorB-lymphoblasticleukemia/lymphomaII.Mature(peripheral)B-cellneoplasmsaB-cellchroniclymphocyticleukemia/smalllymphocyticlymphoma

bB-cellprolymphocyticleukemiacLymphoplasmacyticlymphoma

dSpenicmarginalzoneB-celllymphoma(+/-villouslymphocytes)eNodalmarginalzonelymphoma(+/-monocytoidB-cells)fExtranodalmarginalzoneB-celllymphomaof

mucosa-associatedlymphoidtissue(MALT)type

2021/4/2720WHOClassification,2001

gHairycellleuekmiahPlasmacellmyeloma/plasmacytomaiFollicularlymphoma,folliclecenterjMantlecelllymphomakDiffuselargecellB-celllymphomaMediastinallargeB-celllymphomaIntravascularlargeB-celllymphomaPrimaryeffusionlymphomalBurkitt'slymphoma/Burkitt'scellleukemiaB-cellproliferationsofuncertainmalignantpotential

Lymphomatoidgranulomatosis

Post-transplantlymphoproliferativedisorder

2021/4/2721WHOClassification,2001T-CellandNaturalKillerCellNeoplasmsI.PrecursorTcellneoplasm:

a.PrecursorT-lymphoblasticlymphoma/leukemia

b.BlasticNKlymphoma2021/4/2722WHOClassification,2001II.Mature(peripheral)TcellandNK-cellneoplasmsaTcellprolymphocyticleukemiabT-cellgranularlymphocyticleukemiacAggressiveNKCellleukemiadAdultTcelllymphoma/leukemia(HTLV1+)eExtranodalNK/T-celllymphoma,nasaltypefEnteropathy-typeT-celllymphomagHepatosplenicgamma-deltaT-celllymphomahSubcutaneouspanniculitis-likeT-celllymphomaiMycosisfungoides/Sezary'ssyndrome2021/4/2723WHOClassification,2001

jPrimaryCutaneousAnaplasticlargecelllymphomaT/nullcellkPeripheralTcelllymphoma,unspecifiedlAngioimmunoblasticTcelllymphomamPrimarySystemicAnaplasticlargecelllymphoma,T/nullcellT-cellproliferationofuncertainmalignantpotential

Lymphomatoidpapulosis2021/4/2724ClinicalFeatures2021/4/2725

HDNHL

Age

Youngpeople

Moreofteninmiddle-agedpeople

Superficiallymphnodes

60～80％

50%

(firstobservedastumefactionofcervicalorsupraclavicularlymphnodes)

Mediastinallymphnodes

50%

20%

Retroperitoneallymphnodes

Seldominvaded

Ofteninvaded

Fever

Oftenoccurred

Seldominearlyperiod

Itchofskin

Oftenoccurredinsomeyoungfemalepatients

Seldomoccurred

Specificskinlesion

Seldomoccurred

OftenoccurredExtranodalsites

Seldomoccurred

Oftenoccurred

(Waldeyer’sring,gastrointestinaltract,bonemarrow,CNS,liver,lung,pleura,bone,skin,kidney)

Wayofdissemination

Metastasistonearbylymphnodes

Inajumpingway

Bonemarrowinvolvement

SeldomoccurredOftenoccurred

Progress

Slower

Faster

Prognosis

Better

Worse

2021/4/2726LabandOtherFindingsPeripheralBloodandBoneMarrowHD：Eosinophilia；R－Scell NHL：Lymphocytosis;ComplicatedleukemiaLDH，β2-MG,Coombstest，globulinRadiologicstudiesChestX-rayCT,MRIRadioactivenuclidePET-CTB-ultrasoundBiopsyImmunophenotypeCytogeneticandMolecularStudies2021/4/2727Diagnosis

Painless,progressivelymphadenectasis→Biopsy

SkinLesion→BiopsyBoneMarrowAspirationandBiopsyBiopsyofOtherSites

DiagnosisandDifferentialDiagnosis2021/4/2728StagingoflymphomaStageDefinitionⅠInvolvementofasinglelymphnoderegionorofasingleextranodalorganorsite(ⅠE)ⅡInvolvementoftwoormorenoderegionsonthesamesideofthediaphragm,orlocalizedinvolvementofanextranodalsiteororgan(ⅡE)andoneormorelymphnoderegionsonthesamesideofthediaphragmⅢInvolvementoflymphnoderegionsonbothsidesofthediaphragmwhichmayalsobeaccompaniedbylocalizedinvolvementofanextranodalorgansorsite(ⅢE)orspleen(Ⅲs)orboth(ⅢsE)ⅣDiffuseordisseminatedinvolvementofoneormoredistantextranodalorganswithorwithoutassociatedlymphnodeinvolvementBsymptomsFever>38℃,nightsweats,and/orweightloss>10%ofbodyinthe6months2021/4/2729Stagingoflymphoma2021/4/2730DifferentialDiagnosisLymphadenectasis：

ChroniclymphadenitisTuberculouslymphadenitisTumournodemetastasesFever：Septicaemia,Tuberculosis,RheumaticdiseaseExtranodalLymphoma:OthermalignanttumerDiagnosisandDifferentialDiagnosis2021/4/2731RadiationTherapy

60Co,acceleratorlinearDosage:30～40GY，3～4WMethods：

Localfieldirradiation(LFI)

MantleorinvertedYirradiationTotalnodalirradiation(TNI)Treatment2021/4/2732TreatmentFields2021/4/2733Indication：HDⅠA,ⅡA：Extendedfieldirradiation(EFI)ⅠB,ⅡB,ⅢA：TNIand/orcombination