TY  - CHAP
M1  - Book, Section
TI  - Pulmonary Langerhans Cell Histiocytosis
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.
Y1  - 2023
N1  - 
T2  - Fishman’s Pulmonary Diseases and Disorders, 6e
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  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/23
UR  - accessmedicine.mhmedical.com/content.aspx?aid=1195006355
ER  -