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1、Received:30March2016Revised:19May2016Accepted:19May2016Publishedon:17June2016DOI:10.1002/pbc.26104PediatricREVIEWBlood&CancerTheAmericanSocietyofPediatricHematology/OncologyLangerhanscellhistiocytosisisaneoplasmandconsequentlyitsrecurrenceisarelapseInmem
2、oryofBobArceciR.MaartenEgeler1SatyendraKatewa2PieterJ.M.Leenen3PeterBeverley4MatthewCollin5FlorentGinhoux6RobertJ.Arceci7,∗BarrettJ.Rollins8,9fortheSteeringCommitteeoftheNikolasSymposium1DivisionofHaematology/Oncology,Depart-mentofPaediatrics,TheHospital
3、forSickChil-Abstractdren/UniversityofToronto,Toronto,Ontario,Langerhanscellhistiocytosis(LCH)remainsapoorlyunderstooddisorderwithheterogeneousCanadaclinicalpresentationscharacterizedbyfocalordisseminatedlesionsthatcontainexcessive2DepartmentofPediatricHe
4、matology/OncologyCD1a+langerin+cellswithdendriticcellfeaturesknownasLCHcells.Twoofthemajorques-&BMT,SoniManipalHospital,MainSikarRoad,tionsinvestigatedoverthepastcenturyhavebeen(i)theoriginofLCHcellsand(ii)whetherLCHSector5,Jaipur,Rajasthan,India3isprima
5、rilyanimmunedysregulatorydisorderoraneoplasm.CurrentopinionisthatLCHcellsDepartmentofImmunology,ErasmusMedicalCenter,Rotterdam,TheNetherlandsarelikelytoarisefromhematopoieticprecursorcells,althoughthestageofderailmentandsite4Abingdon,UnitedKingdomoftrans
6、formationremainunclearandmayvaryinpatientswithdifferentextentofdisease.Over5DepartmentofHaematologicalSciences,Insti-theyears,evidencehasprovidedtheviewthatLCHisaneoplasm.ThedemonstrationofclonalitytuteofCellularMedicine,NewcastleUniversity,ofLCHcells,in
7、sufficientevidencealoneforneoplasia,isnowbolsteredbyfindingdriversomaticNewcastleUponTyne,UnitedKingdommutationsinBRAFinupto55%ofpatientswithLCH,andactivationoftheRAS-RAF-MEK-ERK6SingaporeImmunologyNetwork(SIgN),Agency(whereMEKandERKaremitogen-activatedpro
8、teinkinaseandextracellularsignal-regulatedforScience,TechnologyandResearch(A*STAR),Singapore,138648kinase,respectively)pathwayinnearly100%ofpatientswithLCH.Herein,wereviewtheevidence7DepartmentofChildHealth,Universityoftha
