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1、IntJClinExpPathol(2008)1,396-402
www.ijcep.com/IJCEP710002ReviewArticleFibrosingCholestaticHepatitis:Clinicopathologic
Spectrum,DiagnosisandPathogenesisShu-YuanXiao1,LiangLu1andHanlinL.Wang21DepartmentsofPathologyandofInternalMedicine,andInstituteforHumanInfectionsandImmunity,Univ
2、ersity
ofTexasMedicalBranch,Galveston,Texasand2DepartmentofPathologyandLaboratoryMedicine,Cedars-Sinai
MedicalCenter,LosAngeles,CaliforniaReceived2Oct2007;acceptedandavailableonline30Oct2007Abstract:Fibrosingcholestatichepatitis(FCH)isarapidlyprogressive,sometimesfatalformofliveri
3、njury.Though
originallyreportedinlivertransplantrecipientswithrecurrenthepatitisB,ithasnowbeenrecognizedfrequentlyin
chronichepatitisBorCpatientswhoareunderimmunosuppression.Thehistopathologichallmarksintheliver
includemarkedhepatocyticinjury,severecholestasis,andperiportalandperi
4、cellularfibrosis.Thepathogenesisis
largelyunknown.TheaimofthisreviewistodescribethespectrumofclinicalconditionsinwhichFCHoccurs,
commonhistopathologicfindings,featuresuniquetocertainunderlyingdiseases,factorstobeconsideredin
differentialdiagnosis,andourcurrentunderstandingofpathog
5、enesisofthisdisease.KeyWords:Viralhepatitis,hepatitisBvirus,hepatitisCvirus,livertransplantation,pathogenesisIntroductionTheterm“fibrosingcholestatichepatitis”(FCH)wasfirstcoinedin1991todescribeasevere,fulminantformofrecurrenthepatitisBinlivertransplantrecipients[1,2],althoughcase
6、swithsimilarclinicopathologicpresentationshadbeenrecordedearlier[3,4].Ithasalsobeendescribedunderothernames,suchas“fibrosingcytolyticliverfailure”[5]and“fibroviralhepatitis”[6].Clinically,FCHischaracterizedbyrapidandprogressivedeteriorationingraftfunctions,evidencedbyseverejaundic
7、e,coagulopathy,encephalo-pathyanddeathwithin4-6weeksofonset[2].Laboratorytestsusuallyshowelevationofserumbilirubinlevel,prolongedprothrombintime,andmildtomoderateincreaseinserumtransaminases.Theinitiallyreportedcaseswereallrapidlyprogressiveandfatal,andwereassociatedwithhighlevels
8、ofviralantigenexpressioninthelive
