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Cell(s) of Origin of Langerhans Cell Histiocytosis

Lookup NU author(s): Professor Matthew CollinORCiD, Dr Venetia BigleyORCiD

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Abstract

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.

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Author(s): Collin M, Bigley V, McClain KL, Allen CE

Publication type: Article

Publication status: Published

Journal: Hematology/Oncology Clinics of North America

Year: 2015

Volume: 29

Issue: 5

Pages: 825-838

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.

URL: http://dx.doi.org/10.1016/j.hoc.2015.06.003

DOI: 10.1016/j.hoc.2015.06.003

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