血液病英文ppt

TheDiseasesofHematopoieticandlymphoidsystems Structureandfunction Hematopoieticandlymphoidsystemiscomposedofmyeloidtissue bonemarrow andlymphoidtissues thymus spleen lymphnodesandextranodelymphoidtissues ThethymusandbonemarrowareoftentermedcentrallymphoidtissuesTheremainingorgansareactivelyinvolvedintheimmuneresponse andconstitutetheperipherallymphoidtissue Diseases InfectionandreactiveproliferationsThelymphoidneoplasmasMyeloidneoplasmsHistocyticneoplasms Lymphoidneoplasms Alllymphoidneoplasmsarederivedfromasingletransformedcellandarethereforemonoclonal Lymphoidneoplasmsareagroupoftumorsthattheirclinicalmanifestationsandbehaviorvarywidely Theyareclassifiedasnon Hodgkin slymphoma NHLs andHodgkins slymphomabasedonthefeaturesoftumorcells NHLsaccountforabout70 to80 ofallmalignantlymphomasinourcountry Classificationoflymphoidneoplasms Theaimofclassificationistoidentifyhomogeneroussubgroupsthatbehaveinapredictableway Thelymphoidneoplasmsarenamedaccordingtothenormalcelltheymostcloselyresemble WHOclassifications 2000 intable9 1 page276 1948Willis Nowhereinpathologyhasthechaosofnamessocloudedclearconceptsasinthesubjectoflymphoidtumors 分类 NHL分类方法多 分型繁杂 Classification Threemajorcategories 1TumorsofB cells2TumorsofT cellsandNK cells3Hodgkin slymphoma Non Hodgkin slymphoma Etiology 1 1 virusMurine feline avianleukemiasretrovirusesadultTcelllymphoma leukemiaHTLV 1Burkitt slymphomaEBV Etiology 2 2 oncogenesBurkitt slymphoma t 8 14 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 Burkitt slymphoma starry sky Burkitt slymphoma mitoses PeripheralT celllymphoma ItisrelativelycommoninAsiacomparedwithAmericaandEurope ItisassociatedwithhumanT cellleukemia lymphomavirus HTL1 Themorphologyisvariableinsizeandshapesofcells Thesamefeaturessuchasthearchitecturesoflymphnodereplacedbythetumorcells CD2 CD3 CD5 Lymphoblastic T convoluted Hodgkin slymphoma Hodgkin slymphoma alsocalledHodgkin sdisease isaprimarymalignanttumoroflymphoidtissues ItischaracterizedbythepresenceofReed Sternberg RS cellsintheinvolvedtissues Itaccountsfor15 ofalllymphomasandshowsapeakageincidenceinthethirdandfourthdecades ThereasonsitseparatedfromNHLs 1MorphologicallycharacterizedbythepresenceofdistinctiveR Scellsadmixedwithavariableinfiltrateofreactive nonmalignantinflammatorycells 2Itisoftenassociatedwithsomewhatdistinctiveclinicalfeatures includingsystemicmanifestations suchasfever 3Itsstereotypicpatternofspreadallowsittobetreateddifferentlythanmostotherlymphoidneoplasm ClassificationofHodgkin slymphoma Itisclassifiedbasedontheappe