cell nevus syndrome manifestations in noncaucasian 在非白种人的基底细胞痣综合征表现课件

cell nevus syndrome manifestations in noncaucasian 在非白种人的基底细胞痣综合征表现课件

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时间:2018-10-19

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1、MorningReportAnneLachiewiczSeptember22,2009Broncholitisobliteransorganizingpneumonia(BOOP)CryptogenicorganizingpneumoniaInflammatorylungdiseaseIntraluminalorganizingfibrosisindistalairspaces(bronchioles,alveolarducts,andalveoli)ReversiblefibrosisDxbylungbx.VATpreferredtotransbronch

2、ialbxtoobtainsufficienttissuetoexcludeotherdiagnoseswithoverlappingfeaturesClassificationofBOOPIdiopathic(mostcommon)RapidlyprogressiveFocalnodularPostinfectionDrug-related(casereports)RheumatologicorconnectivetissuerelatedImmunologicdisorderOrgantransplantation(lung,bonemarrow)Rad

3、iotherapy(breastcancer)EnvironmentalexposuresMiscellaneousIntra-alveolarstagesFibrinoidinflammatorycellclusters,fibrinbandswithcells(esp.lymphocytes)Fibroinflammatorybuds,fibroblastsmigrate,proliferate&developMaturefibroticbuds:concentricringsoffibroblastsalternatingwithlayersofcon

4、nectivetissue(mainlycollagenbundles,fewinflammatorycells(usuallyinthecenterofthebuds)MildinterstitialchronicinflammationinalveolarwallswithreactivetypeIIcellsIncreasedfoamymacrophagesinthealveoliwithoutbudsHistologicfeaturesCopyrightrestrictionsmayapply.Epler,G.R.ArchInternMed2001;

5、161:158-164.A,IntraluminalorganizationandpolypoidgranulationtissuewithinasmallbronchiolePreservationoflungarchitecturePatchydistribution,rarelyunilateralUniformtemporalappearanceAbsenceofinterstitialfibrosisgranulomasneutrophilsorabscessesnecrosishyalinemembraneseosinophilicinfiltr

6、ationvasculitisFeaturesMales=FemalesMeanage50-60years2xmoreinnon-smokers/ex-smokersFlu-likeillness,malaise,feverPersistentnonproductivecoughProgressivemilddyspneaWeightlossofgreaterthan10pounds(57%)Inspiratoryralesorfocalsparsecrackles25%normalpulmonaryexamNoclubbingClinicalpresent

7、ationStudiesLabs:50%leukocytosis,elevatedESR,CRP,noeosinophiliaPFTs:mild-modrestrictivedefect,only20%obstructive(allformersmokers)BAL:Mixedpattern(mildincreaseinlymphocytes,neutrophils,eosinophils)Imaging:3mainpatternsofCOPTypical–multiplealveolaropacities,usuallybilateral,peripher

8、al,andoftenmigratory,mayse

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