血液病英文课件

TheDiseasesofHematopoieticandlymphoidsystemsStructureandfunctionHematopoieticandlymphoidsystemiscomposedofmyeloidtissue(bonemarrow)andlymphoidtissues(thymus,spleen,lymphnodesandextranodelymphoidtissues).ThethymusandbonemarrowareoftentermedcentrallymphoidtissuesTheremainingorgansareactivelyinvolvedintheimmuneresponse,andconstitutetheperipherallymphoidtissue.DiseasesInfectionandreactiveproliferationsThelymphoidneoplasmasMyeloidneoplasmsHistocyticneoplasmsLymphoidneoplasmsAlllymphoidneoplasmsarederivedfromasingletransformedcellandarethereforemonoclonal.Lymphoidneoplasmsareagroupoftumorsthattheirclinicalmanifestationsandbehaviorvarywidely.Theyareclassifiedasnon-Hodgkin’slymphoma(NHLs)andHodgkins’slymphomabasedonthefeaturesoftumorcells.NHLsaccountforabout70%to80&ofallmalignantlymphomasinourcountry.ClassificationoflymphoidneoplasmsTheaimofclassificationistoidentifyhomogeneroussubgroupsthatbehaveinapredictableway.Thelymphoidneoplasmsarenamedaccordingtothenormalcelltheymostcloselyresemble.WHOclassifications(2000)intable9-1(page276).1948Willis:“Nowhereinpathologyhasthechaosofnamessocloudedclearconceptsasinthesubjectoflymphoidtumors”分类:NHL分类方法多，分型繁杂ClassificationThreemajorcategories:1TumorsofB-cells2TumorsofT-cellsandNK-cells3Hodgkin’slymphomaNon-Hodgkin’slymphomaEtiology(1)1.virusMurine、feline、avianleukemias

retrovirusesadultTcelllymphoma-leukemia

HTLV-1Burkitt’slymphoma

EBVEtiology(2)2.oncogenesBurkitt’slymphoma:t(8;14)myc-Igfollicularlymphoma:t(14;18)bcl-2-IgCLL:Philadelphiachromosomet(9,22)c-abl,bcrEtiology(3)3.Autoimmune&immunodeficienyDiseaseeg.Receptoroftransplantedorgans.AIDSpatientslymphomaLeukemia/LymphomaThesearehigh-gradeNHLscomposedofdiffusesheetsofmedium-sizelymphoidcells.TheymaybeB-orT-celllineage.Theseaggressivetumorsaffectpredominantlyinchildren,accountingfor80%ofchildhoodleukemia.Thepre-B-celllymphomamainlyaffectschildren,butthePre-T-celltumorsmainlyaffectadolescentmales.MorphologyMircoscopically,thelymphnodesaffectedbyneoplasticcellsarereplacedbysmalltomedium-sizedblastcellswithscantcytoplasmandinconspicuousnucleoli.Inbloodsmearslide,thenucleioflymphoblastswithWright-Giemsastainingshowsomewhatcoarseandclumpedchromatinandoneortwonucleoli.BloodandbonemarrowchangesInperipheralblood,thewhitecellcountisusuallyincreased.sometimesmorethan100,000/ul.Anemiaisalmostpresent.Theplateletcountisususllydepressedtolessthan100,000/ul.Bonepainandtendernessresultfrommarrowexpansionwithinfiltrationofthesubperiosteum.Splenomegaly.ImmunophenotypeandkaryotypeTdTpresentinmorethan95%ofcasesCD19(Bcellmarker)CD2(Tcellmarker)ClinicalfeaturesThemanifestationsinthesediseasesaresimilartothatofAML.Anemia,hemorrhageandinfectionaswellasrelatedsymptomsChroniclymphocyticleukemia(CLL)/smalllymphocyticlymphoma(SLL)Infact,smalllymphocticlymphomaandchroniclymphocyticleukemiaarethevirtuallyidenticaltumors.Itisadiseaseaffectingpersonsolderthan50yearsofage.Mostpatientsareleukemicatthetimeofdiagnosis.MorphologyThelymphnodesarereplacedbysheetsofmaturelymphocytes,whichareround,smallcompactwithdark-stainingroundnuclei,scantycytoplasmanduniforminshapeandsize,andscatteredill-definedfocioflargecellstermedprolymphocytes.Thefociofmitoticactiveprolymphocytesarecalledproliferationcenters,whichareusefulforCLL/SLLindiagnosis.ImmunophenotyeandkaryotypeTheneoplasticcellsexpressB-cellmarkers,suchasCD19,CD20,CD23,surfaceimmunoglobulin(e.g.IgM,IgG).ClinicalfeaturesCLL/SLLisoftenasymptomatic.manycasesarediagnosedasaresultofroutinebloodtestsorclinicalexaminationforotherreasons.Thesymptomarenonspecific,includingeasyfatigue,weightlossandanorexia.Theprognosisisgoodandthepatientswiththesediseasesmaysurvivefor10yearsormore.Themediansurvivaltimeis4to6years.DiffuseFollicularlymphomaItisatumorderivedfromgerminalcenterB-cells,characterizedbyanodularorfolliculararchitecture.ItisoneofthecommonesttypeofNHLs.Itaccountsfor10%to40%ofNHLs.Lateadultlifeisapeakageincidence.ImmunophenotypeandkaryotypeCD19,CD20.CD10,BCLprotein.Mostofthecaseshavespecificchromosometranslocationinvolvingtheimmunoglobulinheavychainpromoterregionofchromosome14andtheanti-apoptoticgeneBCL12onchromosome18(t14,18)(q32,q21).

FollicularCD20CD3DiffuselargeB-celllymphomaAdiffusegrowthpattern.Occursmainlyinolderpatients(medianageabout60year)ItischaracterizedbyadiffuseoutgrowthoflargeB-cells,whichmaydisplaycentroblasticofimmunblasticcytology.CD19,CD20,CD79aandIgMDiffuselargecell,centroblasticCBIBT/HRALCCD30CD20CD3BurkittlymphomaAdistinctivetypeofBcelllymphoma.Itisdndemicinpara-Africaandoccurmuchlesscommonlyinotherregions.Itmainlyaffectchildrenandadolescents.ItisassociatedwithEBVinfectionandmalaria.ItexpressCD10,CD19.Burkitt’slymphoma,starry-skyBurkitt’slymphoma,mitosesPeripheralT-celllymphomaItisrelativelycommoninAsiacomparedwithAmericaandEurope.ItisassociatedwithhumanT-cellleukemia/lymphomavirus(HTL1)Themorphologyisvariableinsizeandshapesofcells.Thesamefeaturessuchasthearchitecturesoflymphnodereplacedbythetumorcells.CD2,CD3,CD5Lymphoblastic(T),convolutedHodgkin’slymphomaHodgkin’slymphoma(alsocalledHodgkin’sdisease)isaprimarymalignanttumoroflymphoidtissues.ItischaracterizedbythepresenceofReed-Sternberg(RS)cellsintheinvolvedtissues.Itaccountsfor15%ofalllymphomasandshowsapeakageincidenceinthethirdandfourthdecades.ThereasonsitseparatedfromNHLs1MorphologicallycharacterizedbythepresenceofdistinctiveR-Scellsadmixedwithavariable