血液病英文ppt

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