简介：Objective:Langerhanscellhistiocytosis(LCH)hasbeenwelldescribedonlyinchildren.Weanalyzedthecharacteristics,reactivation,andoutcomeofLCHinacohortof55patientsacrossallages.Methods:WereviewedtherecordsofallpatientswithLCHtreatedatasingleinstitutebetweenJan.1974andMay1998.Results:The55patientswere2to67yearsofage(median,31years)atthetimeofdiagnosis,and85.5%weremale.Fortypatients(72.7%)hadsingle-systemLCH;Fifteen(27.3%)hadmultisystemdisease.Theheadandneckwasthemostfrequenttumorsite(63.6%).LCHwasnotfoundinorgansatriskofinvolvement(liver,spleen,bonemarrow,andlungs).Thefrequencyofbonyinvasion(23.6%overall)differedsignificantlyaccordingtoage(15years(66.7%)vs.Age>15years(11.6%)(P=0.0005).Atamedianfollow-upof12years,nopatientdiedofLCH.The5,10-yearsurvivalestimateswere100%.The5,10-yeardisease-freesurvivalestimateswere70.9%and58.4%.The5-yeardisease-freesurvivalestimatewas58.3%forage(15yearsvs.74.4%forage>15years(P=0.83)and75%forsingle-systemdiseasevs.60%formultisystemdisease(P=0.13).LCHwasreactivatedin43.6%ofpatients,withamedianof14months(range,2-180months).Threepatientswithrecurrentdiseaseexperiencedspontaneousremission.Atthetimeofthemostrecentfollow-up,23.6%ofsurvivorshadactivedisease.Conclusion:LCHisnotfoundexclusivelyinchildrenandadolescents.Thefrequencyofboneinvasionisinverselyrelatedtoage.Reactivationisverycommonregardlessofthetypeoftreatment,buttheprognosisisgenerallygood.