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Lookup NU author(s): Professor Matthew Collin,
Dr Venetia Bigley
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Langerhans cell histiocytosis (LCH) is a heterogeneous disease characterized by common histology of inflammatory lesions containing Langerin(+) (CD207) histiocytes. Emerging data support a model in which MAPK activation in self-renewing hematopoietic progenitors may drive disseminated high-risk disease, whereas MAPK activation in more differentiated committed myeloid populations may induce low-risk LCH. The heterogeneous clinical manifestations with shared histology may represent the final common pathway of an acquired defect of differentiation, initiated at more than one point. Implications of this model include re-definition of LCH as a myeloid neoplasia and re-focusing therapeutic strategies on the cells and lineages of origin.
Author(s): Collin M, Bigley V, McClain KL, Allen CE
Publication type: Article
Publication status: Published
Journal: Hematology/Oncology Clinics of North America
Print publication date: 01/10/2015
Online publication date: 20/08/2015
ISSN (print): 0889-8588
ISSN (electronic): 1558-1977
Publisher: W.B. Saunders Co.
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