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CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions.

Kenneth L McClainJennifer PicarsicRikhia ChakrabortyDaniel ZinnHoward LinHarshal AbhyankarBrooks ScullAlbert ShihKaren Phaik Har LimOlive EcksteinJoseph LubegaTricia L PetersWalter OleaThomas BurkeNabil AhmedM John HicksBrandon TranJeremy JonesRobert DauserMichael JengRobert BaiocchiDeborah SchiffStanton GoldmanKenneth M HeymHarry WilsonBenjamin CarcamoAshish KumarCarlos Rodriguez-GalindoNicholas S WhipplePatrick CampbellGeoffrey MurdochJulia KoflerSimon HealesMarian MaloneRandy WoltjerJoseph F QuinnPaul OrchardMichael C KruerRonald JaffeMarkus G ManzSergio A LiraD Williams ParsonsMiriam MeradTsz-Kwong ManCarl E Allen
Published in: Cancer (2018)
In LCH-ND patients, BRAFV600E+ cells in PBMCs and infiltrating myeloid/monocytic cells in the brain is consistent with LCH-ND as an active demyelinating process arising from a mutated hematopoietic precursor from which LCH lesion CD207+ cells are also derived. Therapy directed against myeloid precursors with activated MAPK signaling may be effective for LCH-ND. Cancer 2018;124:2607-20. © 2018 American Cancer Society.
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