病理学课件 15-淋巴.ppt

OrgansortissuesBonemarrowLymphnodeSpleenThymus扁桃体、咽淋巴扁桃体、咽淋巴环肠道淋巴道淋巴组织胃、甲状腺、皮肤胃、甲状腺、皮肤M/306 cmx4 cmF/52,左侧鼻翼小结节，按炎症治疗无效CD3骨髓涂片骨髓涂片骨髓活检骨髓活检BenignpathologicalchangesIndolentlymphomainlymphnodeAggressivelymphomainlymphnodeLeukemiaAnti-inflammatorytreatmentAnti-tumortherapyLymphoidneoplasms（malignantlymphoma）Definition:恶性肿瘤恶性肿瘤肿瘤细胞类似于各转化阶段的淋巴细胞及其前体细胞淋巴细胞及其前体细胞包括包括 lymphoma（淋巴瘤（淋巴瘤）lymphoidleukemia（淋巴细胞白血病（淋巴细胞白血病）Plasmacellproliferative（浆细胞疾病）（浆细胞疾病） Lymphoma Hodgkin lymphoma , HL Non-Hodgkin lymphoma , NHLT and NK cell neoplasmsB cell neoplasms Hodgkin Lymphoma , HL( Hodgkins disease, HD) 霍奇金淋巴瘤霍奇金淋巴瘤/病病ThomasHodgkin(1798-1866)ThomasHodgkin(1798-1866)Epidemiology10%-20%10%-20%ofallMLChina9.4Ageandgender双峰曲双峰曲线：15152727岁5050岁Site： Lymphnode:cervixetcextranode：spleenClinicalfeaturesPrimary:lymphadenopathySystemicsymptomBSymptom:fever，Nightsweat，Weightloss，ItchyskinGrossLymphnodeSpleen MorphologyBackgroundofinflammatorycellsDistinctiveneoplasticcellsDestroyofstructureClassical RS cellHodgkin cellLacunar cellPopcorn CellPleomorphicRS cellMirror image cellCD15 (+)CD30 (+)ImmunophenotypeofHodgkincellsCD30 active lymphocyte nearly 100% +CD15 monocyte about 80% + CD20 B cell popcorn cell +Lymphocyte-richHL,LRHL富于淋巴细胞型富于淋巴细胞型Clinicalfeature5% of all HL5 years survival rate60 Transformed into mixed cellularity HL Clinicalfeature40-70% of all HLYong womenCervical，Supraclavicular and Mediastinal lymph nodeGood prognosisNodularsclerosisHL,NSHL结节硬化型结节硬化型MixedcellularityHL,MCHL混合细胞型混合细胞型Clinicalfeature20% of all classical HLChinese people more commonMore common in men5 years survival rate39Transformed into lymphocyte depletion HL 淋巴细胞消减型淋巴细胞消减型LymphocytedepletionHL,LDHLImmunophenotypeRScell:CD30(+)CD15(+)Bcellmarker(-)ClinicalfeaturerareOldermenSymptoms，Poorprognosis，5yearsSurvivalrate0，Averagesurvival4-42monthsClinicalfeature:Youngmale：35岁，岁，M:F2.5:1Clinicalfeature：notobviousPrognosis：10yearssurvivalrate802-6%ofallHL3-5%BcelllymphomaNodularlymphocytepredominantHL,NLPHL结节性淋巴性淋巴细胞胞为主型主型Immunotype:CD30(-)CD15(-)Bcellmarker(+)Genetype:clonalIggenerearrangmentHodgkin LymphomaNodularlymphocytepredominantHL,NLPHL结节性淋巴性淋巴细胞胞为主型主型Non-Hodgkinlymphoma,NHL非霍奇金淋巴瘤非霍奇金淋巴瘤80-90ofalllymphomaLymphnode（2/3）orextranodaltissue（1/3）Clinicalfeature ：complicatedMorphology：predominanttumorcellmonomorphicLymphoma versus Lymphoid leukemia非实体瘤非实体瘤原发于骨髓，累及外周血原发于骨髓，累及外周血肿肿 块块淋巴结内或结外器官淋巴结内或结外器官白血病样象白血病样象累及外周血累及外周血淋巴瘤组织象淋巴瘤组织象累及淋巴结和结外组织累及淋巴结和结外组织Since both solid and circulating phases are present in many lymphoid neoplasms, the distinction between them is artificial.OriginprecursorBcell/TcellEpidemiologyB-LB：children（10years），），LeukaemiaT-LB：adolescent，LymphomaB- and T-cell lymphoblastic lymphoma /leukemia B/T淋巴母细胞淋巴瘤淋巴母细胞淋巴瘤/白血病白血病 ClinicalfeatureHighaggressiveBonemarrowinvolvementAnemia,bleed,infection,etcLymphadenopathyhepatomegalysplenomegalyLBAMLnMorphology肿瘤性淋巴母细胞的弥漫性增生浸润肿瘤性淋巴母细胞的弥漫性增生浸润Lymphblastic lymphomaImmunophenotype TdT 95（+）T cell marker：CD3、CD2B cell marker：CD19、CD20、CD79aCytogenetic abnormality 90delete translocation hypodiploidhyperdiploid trisomyTreatmentmultiagentChemotherapy 90 complete remission(CR)2/3 curableTdTDiffuselargeBcelllymphoma,DLBCL弥漫性大弥漫性大B细胞淋巴瘤细胞淋巴瘤Definition a diffuse proliferation of largeneoplasticBlymphoidcellswithnuclearsizeequal to or exceeding normal macrophagenucleiormorethantwicethesizeofnormallymphocyte.origin: germinal center B-cell or active B-cellEpidemiology The commonest NHL: 30-40% of adult NHL in western countries, even higher in developing countriesAge : the range is broad, the median age: 7th decadeSex : more common in males than femalesAggressive, but potentially curable with multiagent chemotherapyClinicalfeaturesA rapidly enlarging, often symptomaticmass at a single nodal or extranodalsite.Morphologylargeneoplastic,diffuseproliferation ImmunophenotypePan-B(+)：CD19CD20CD79a30-50%BCL2(+),t(14;18)BCL6(+)Cytogeneticabnormality3q27Bcl-6,8q24C-mycTreatmentmultiagentchemotherapyTargeteddrugs：Rituximab美罗华美罗华ExtranodalmarginalzonelymphomaOfmucosa-associatedlymphoidtissue粘膜相关淋巴组织淋巴瘤粘膜相关淋巴组织淋巴瘤Smallcell、Bcell、indolentPrognosisisgoodGastricMALTomaHPinfectionHPEradicationtherapy,RecoveryoftheMALTomaBeforethetreatment 2weeksaftertreatment 10monthsaftertreatment12 mo post CHOP 21 mo post CHOP6 mo post Hp Rx (27 mo post CHOP)H pyloriSB: Female aged 41t( 1;14)(p22;q32)/IgH-BCL10 t(14;18)(q32;q21)/IgH- MALT1t(11;18)(q21;q21)/API2-MALT1 T/NKcelllymphomaT/NK细胞淋巴瘤细胞淋巴瘤ClinicalfeaturesAsia“Chineselymphoma”ExtranodalinvolvementnoseHighaggressiveMorphologyAngiocentricandangiodestructivegrowthpatternNecrosisPleomorphologyImmunophenotypeTcell：CD2CD3NKcell：CD56Cytotoxicmarker:GramBTIA-1EBvirus80GeneticsTCR（-）CD3CD56EBV-EBERSkin，indolen，TcelllymphomaClinicalfeaturesincidence ：0.5%Morecommoninoldermen( (6464岁岁, ,1 1.86:1).86:1)RecurringrashLatediffusiontointernalorgansandlymphnodesTumorcellsinbloodSzarySyndromeMycosisfungoidesMF蕈样霉菌病蕈样霉菌病PATCHPLAQUETUMORMorphologyLymphocyteswithirregularnucleiPautriermicroabcesses Immunopheno。