RT Book, Section
A1 Harari, Sergio
A1 Elia, Davide
A1 Moss, Joel
A1 Tazi, Abdellatif
A2 Grippi, Michael A.
A2 Antin-Ozerkis, Danielle E.
A2 Dela Cruz, Charles S.
A2 Kotloff, Robert M.
A2 Kotton, Camille Nelson
A2 Pack, Allan I.
SR Print(0)
ID 1195006355
T1 Pulmonary Langerhans Cell Histiocytosis
T2 Fishman’s Pulmonary Diseases and Disorders, 6e
YR 2023
FD 2023
PB McGraw-Hill Education
PP New York, NY
SN 9781260473988
LK accessmedicine.mhmedical.com/content.aspx?aid=1195006355
RD 2024/04/19
AB Histiocytosis  or  histiocytic  disorders  are  characterized  by  abnormal  function  and  accumulation  or  differentiation  of  specific  cells,  thought  to  be  derived  from  dendritic  cells  (DCs)  or  the  monocyte–macrophage  lineage.1  Among  this  group  of  diseases,  Langerhans  cell  histiocytosis  (LCH)  is  the  most  common  and  is  characterized  by  an  activation  of  the  MAPK-ERK  pathway,  driven  by  various  molecular  abnormalities  in  this  pathway,  particularly  the  canonical  BRAFV600E  mutation  in  about  half  of  LCH  lesions.2,3  Importantly,  the  cell-specific  gene  expression  signature  in  Langerin  (CD207)-positive  cells  within  specific  lesions  has  shown  that  LCH  cells  are  more  consistent  with  immature  myeloid  dendritic  cell  precursors  than  with  Langerhans  cells.4