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内科学淋巴瘤邹善华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
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