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血液病英文,1,TheDiseasesofHematopoieticandlymphoidsystems,血液病英文,2,Structureandfunction,Hematopoieticandlymphoidsystemiscomposedofmyeloidtissue(bonemarrow)andlymphoidtissues(thymus,spleen,lymphnodesandextranodelymphoidtissues).ThethymusandbonemarrowareoftentermedcentrallymphoidtissuesTheremainingorgansareactivelyinvolvedintheimmuneresponse,andconstitutetheperipherallymphoidtissue.,血液病英文,3,血液病英文,4,血液病英文,5,血液病英文,6,血液病英文,7,Diseases,InfectionandreactiveproliferationsThelymphoidneoplasmasMyeloidneoplasmsHistocyticneoplasms,血液病英文,8,Lymphoidneoplasms,Alllymphoidneoplasmsarederivedfromasingletransformedcellandarethereforemonoclonal.Lymphoidneoplasmsareagroupoftumorsthattheirclinicalmanifestationsandbehaviorvarywidely.Theyareclassifiedasnon-Hodgkinslymphoma(NHLs)andHodgkinsslymphomabasedonthefeaturesoftumorcells.NHLsaccountforabout70%to8014)myc-Igfollicularlymphoma:t(14;18)bcl-2-IgCLL:Philadelphiachromosomet(9,22)c-abl,bcr,血液病英文,17,Etiology(3),3.Autoimmune&immunodeficienyDiseaseeg.Receptoroftransplantedorgans.AIDSpatients,血液病英文,18,lymphoma,血液病英文,19,Leukemia/Lymphoma,Thesearehigh-gradeNHLscomposedofdiffusesheetsofmedium-sizelymphoidcells.TheymaybeB-orT-celllineage.Theseaggressivetumorsaffectpredominantlyinchildren,accountingfor80%ofchildhoodleukemia.Thepre-B-celllymphomamainlyaffectschildren,butthePre-T-celltumorsmainlyaffectadolescentmales.,血液病英文,20,Morphology,Mircoscopically,thelymphnodesaffectedbyneoplasticcellsarereplacedbysmalltomedium-sizedblastcellswithscantcytoplasmandinconspicuousnucleoli.Inbloodsmearslide,thenucleioflymphoblastswithWright-Giemsastainingshowsomewhatcoarseandclumpedchromatinandoneortwonucleoli.,血液病英文,21,Bloodandbonemarrowchanges,Inperipheralblood,thewhitecellcountisusuallyincreased.sometimesmorethan100,000/ul.Anemiaisalmostpresent.Theplateletcountisususllydepressedtolessthan100,000/ul.Bonepainandtendernessresultfrommarrowexpansionwithinfiltrationofthesubperiosteum.Splenomegaly.,血液病英文,22,Immunophenotypeandkaryotype,TdTpresentinmorethan95%ofcasesCD19(Bcellmarker)CD2(Tcellmarker),血液病英文,23,Clinicalfeatures,ThemanifestationsinthesediseasesaresimilartothatofAML.Anemia,hemorrhageandinfectionaswellasrelatedsymptoms,血液病英文,24,Chroniclymphocyticleukemia(CLL)/smalllymphocyticlymphoma(SLL),Infact,smalllymphocticlymphomaandchroniclymphocyticleukemiaarethevirtuallyidenticaltumors.Itisadiseaseaffectingpersonsolderthan50yearsofage.Mostpatientsareleukemicatthetimeofdiagnosis.,血液病英文,25,Morphology,Thelymphnodesarereplacedbysheetsofmaturelymphocytes,whichareround,smallcompactwithdark-stainingroundnuclei,scantycytoplasmanduniforminshapeandsize,andscatteredill-definedfocioflargecellstermedprolymphocytes.Thefociofmitoticactiveprolymphocytesarecalledproliferationcenters,whichareusefulforCLL/SLLindiagnosis.,血液病英文,26,Immunophenotyeandkaryotype,TheneoplasticcellsexpressB-cellmarkers,suchasCD19,CD20,CD23,surfaceimmunoglobulin(e.g.IgM,IgG).,血液病英文,27,血液病英文,28,Clinicalfeatures,CLL/SLLisoftenasymptomatic.manycasesarediagnosedasaresultofroutinebloodtestsorclinicalexaminationforotherreasons.Thesymptomarenonspecific,includingeasyfatigue,weightlossandanorexia.Theprognosisisgoodandthepatientswiththesediseasesmaysurvivefor10yearsormore.Themediansurvivaltimeis4to6years.,血液病英文,29,Diffuse,血液病英文,30,血液病英文,31,Follicularlymphoma,ItisatumorderivedfromgerminalcenterB-cells,characterizedbyanodularorfolliculararchitecture.ItisoneofthecommonesttypeofNHLs.Itaccountsfor10%to40%ofNHLs.Lateadultlifeisapeakageincidence.,血液病英文,32,Immunophenotypeandkaryotype,CD19,CD20.CD10,BCLprotein.Mostofthecaseshavespecificchromosometranslocationinvolvingtheimmunoglobulinheavychainpromoterregionofchromosome14andtheanti-apoptoticgeneBCL12onchromosome18(t14,18)(q32,q21).,血液病英文,33,Follicular,血液病英文,34,血液病英文,35,血液病英文,36,CD20,CD3,血液病英文,37,血液病英文,38,DiffuselargeB-celllymphoma,Adiffusegrowthpattern.Occursmainlyinolderpatients(medianageabout60year)ItischaracterizedbyadiffuseoutgrowthoflargeB-cells,whichmaydisplaycentroblasticofimmunblasticcytology.CD19,CD20,CD79aandIgM,血液病英文,39,Diffuselargecell,centroblastic,血液病英文,40,血液病英文,41,CB,IB,T/HR,ALC,CD30,CD20,CD3,血液病英文,42,Burkittlymphoma,AdistinctivetypeofBcelllymphoma.Itisdndemicinpara-Africaandoccurmuchlesscommonlyinotherregions.Itmainlyaffectchildrenandadolescents.ItisassociatedwithEBVinfectionandmalaria.ItexpressCD10,CD19.,血液病英文,43,Burkittslymphoma,starry-sky,血液病英文,44,Burkittslymphoma,mitoses,血液病英文,45,血液病英文,46,血液病英文,47,PeripheralT-celllymphoma,ItisrelativelycommoninAsiacomparedwithAmericaandEurope.ItisassociatedwithhumanT-cellleukemia/lymphomavirus(HTL1)Themorphologyisvariableinsizeandshapesofcells.Thesamefeaturessuchasthearchitecturesoflymphnodereplacedbythetumorcells.CD2,CD3,CD5,血液病英文,48,Lymphoblastic(T),convoluted,血液病英文,49,Hodgkinslymphoma,Hodgkinslymphoma(alsocalledHodgkinsdisease)isaprimarymalignanttumoroflymphoidtissues.ItischaracterizedbythepresenceofReed-Sternberg(RS)cellsintheinvolvedtissues.Itaccountsfor15%ofalllymphomasandshowsapeakageincidenceinthethirdandfourthdecades.,血液病英文,50,ThereasonsitseparatedfromNHLs,1MorphologicallycharacterizedbythepresenceofdistinctiveR-Scellsadmixedwithavariableinfiltrateofreactive,nonmalignantinflammatorycells.2Itisoftenassociatedwithsomewhatdistinctiveclinicalfeatures,includingsystemicmanifestations,suchasfever.3Itsstereotypicpatternofspreadallowsittobetreateddifferentlythanmostotherlymphoidneoplasm.,血液病英文,51,ClassificationofHodgkinslymphoma,Itisclassifiedbasedontheappearanceoftheabnormalcel
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