血液病英文ppt课件.ppt

TheDiseasesofHematopoieticandlymphoidsystems 1 Structureandfunction Hematopoieticandlymphoidsystemiscomposedofmyeloidtissue bonemarrow andlymphoidtissues thymus spleen lymphnodesandextranodelymphoidtissues ThethymusandbonemarrowareoftentermedcentrallymphoidtissuesTheremainingorgansareactivelyinvolvedintheimmuneresponse andconstitutetheperipherallymphoidtissue 2 3 4 5 6 Diseases InfectionandreactiveproliferationsThelymphoidneoplasmasMyeloidneoplasmsHistocyticneoplasms 7 Lymphoidneoplasms Alllymphoidneoplasmsarederivedfromasingletransformedcellandarethereforemonoclonal Lymphoidneoplasmsareagroupoftumorsthattheirclinicalmanifestationsandbehaviorvarywidely Theyareclassifiedasnon Hodgkin slymphoma NHLs andHodgkins slymphomabasedonthefeaturesoftumorcells NHLsaccountforabout70 to80 ofallmalignantlymphomasinourcountry 8 Classificationoflymphoidneoplasms Theaimofclassificationistoidentifyhomogeneroussubgroupsthatbehaveinapredictableway Thelymphoidneoplasmsarenamedaccordingtothenormalcelltheymostcloselyresemble WHOclassifications 2000 intable9 1 page276 9 1948Willis Nowhereinpathologyhasthechaosofnamessocloudedclearconceptsasinthesubjectoflymphoidtumors 分类 NHL分类方法多 分型繁杂 10 11 12 Classification Threemajorcategories 1TumorsofB cells2TumorsofT cellsandNK cells3Hodgkin slymphoma 13 Non Hodgkin slymphoma 14 Etiology 1 1 virusMurine feline avianleukemiasretrovirusesadultTcelllymphoma leukemiaHTLV 1Burkitt slymphomaEBV 15 Etiology 2 2 oncogenesBurkitt slymphoma t 8 14 myc Igfollicularlymphoma t 14 18 bcl 2 IgCLL Philadelphiachromosomet 9 22 c abl bcr 16 Etiology 3 3 Autoimmune immunodeficienyDiseaseeg Receptoroftransplantedorgans AIDSpatients 17 lymphoma 18 Leukemia Lymphoma Thesearehigh gradeNHLscomposedofdiffusesheetsofmedium sizelymphoidcells TheymaybeB orT celllineage Theseaggressivetumorsaffectpredominantlyinchildren accountingfor80 ofchildhoodleukemia Thepre B celllymphomamainlyaffectschildren butthePre T celltumorsmainlyaffectadolescentmales 19 Morphology Mircoscopically thelymphnodesaffectedbyneoplasticcellsarereplacedbysmalltomedium sizedblastcellswithscantcytoplasmandinconspicuousnucleoli Inbloodsmearslide thenucleioflymphoblastswithWright Giemsastainingshowsomewhatcoarseandclumpedchromatinandoneortwonucleoli 20 Bloodandbonemarrowchanges Inperipheralblood thewhitecellcountisusuallyincreased sometimesmorethan100 000 ul Anemiaisalmostpresent Theplateletcountisususllydepressedtolessthan100 000 ul Bonepainandtendernessresultfrommarrowexpansionwithinfiltrationofthesubperiosteum Splenomegaly 21 Immunophenotypeandkaryotype TdTpresentinmorethan95 ofcasesCD19 Bcellmarker CD2 Tcellmarker 22 Clinicalfeatures ThemanifestationsinthesediseasesaresimilartothatofAML Anemia hemorrhageandinfectionaswellasrelatedsymptoms 23 Chroniclymphocyticleukemia CLL smalllymphocyticlymphoma SLL Infact smalllymphocticlymphomaandchroniclymphocyticleukemiaarethevirtuallyidenticaltumors Itisadiseaseaffectingpersonsolderthan50yearsofage Mostpatientsareleukemicatthetimeofdiagnosis 24 Morphology Thelymphnodesarereplacedbysheetsofmaturelymphocytes whichareround smallcompactwithdark stainingroundnuclei scantycytoplasmanduniforminshapeandsize andscatteredill definedfocioflargecellstermedprolymphocytes Thefociofmitoticactiveprolymphocytesarecalledproliferationcenters whichareusefulforCLL SLLindiagnosis 25 Immunophenotyeandkaryotype TheneoplasticcellsexpressB cellmarkers suchasCD19 CD20 CD23 surfaceimmunoglobulin e g IgM IgG 26 27 Clinicalfeatures CLL SLLisoftenasymptomatic manycasesarediagnosedasaresultofroutinebloodtestsorclinicalexaminationforotherreasons Thesymptomarenonspecific includingeasyfatigue weightlossandanorexia Theprognosisisgoodandthepatientswiththesediseasesmaysurvivefor10yearsormore Themediansurvivaltimeis4to6years 28 Diffuse 29 30 Follicularlymphoma ItisatumorderivedfromgerminalcenterB cells characterizedbyanodularorfolliculararchitecture ItisoneofthecommonesttypeofNHLs Itaccountsfor10 to40 ofNHLs Lateadultlifeisapeakageincidence 31 Immunophenotypeandkaryotype CD19 CD20 CD10 BCLprotein Mostofthecaseshavespecificchromosometranslocationinvolvingtheimmunoglobulinheavychainpromoterregionofchromosome14andtheanti apoptoticgeneBCL12onchromosome18 t14 18 q32 q21 32 Follicular 33 34 35 CD20 CD3 36 37 DiffuselargeB celllymphoma Adiffusegrowthpattern Occursmainlyinolderpatients medianageabout60year ItischaracterizedbyadiffuseoutgrowthoflargeB cells whichmaydisplaycentroblasticofimmunblasticcytology CD19 CD20 CD79aandIgM 38 Diffuselargecell centroblastic 39 40 CB IB T HR ALC CD30 CD20 CD3 41 Burkittlymphoma AdistinctivetypeofBcelllymphoma Itisdndemicinpara Africaandoccurmuchlesscommonlyinotherregions Itmainlyaffectchildrenandadolescents ItisassociatedwithEBVinfectionandmalaria ItexpressCD10 CD19 42 Burkitt slymphoma starry sky 43 Burkitt slymphoma mitoses 44 45 46 PeripheralT celllymphoma ItisrelativelycommoninAsiacomparedwithAmericaandEurope ItisassociatedwithhumanT cellleukemia lymphomavirus HTL1 Themorphologyisvariableinsizeandshapesofcells Thesamefeaturessuchasthearchitecturesoflymphnodereplacedbythetumorcells CD2 CD3 CD5 47 Lymphoblastic T convoluted 48 Hodgkin slymphoma Hodgkin slymphoma alsocalledHodgkin sdisease isaprimarymalignanttumoroflymphoidtissues ItischaracterizedbythepresenceofReed Sternberg RS cellsintheinvolvedtissues Itaccountsfor15 ofalllymphomasandshowsapeakageincidenceinthethirdandfourthdecades 49 ThereasonsitseparatedfromNHLs 1MorphologicallycharacterizedbythepresenceofdistinctiveR Scellsadmixedwithavariableinf