- Title
- Langerhans cell histiocytosis : a malignant myeloid neoplasm or disorder of immune regulation?
- Creator
- Mitchell, Jenee; Kannourakis, George
- Date
- 2021
- Type
- Text; Journal article
- Identifier
- http://researchonline.federation.edu.au/vital/access/HandleResolver/1959.17/178835
- Identifier
- vital:15475
- Identifier
-
https://doi.org/10.1111/apa.15995
- Identifier
- ISBN:0803-5253 (ISSN)
- Abstract
- Langerhans cell histiocytosis (LCH) is characterised by lesions containing LCH cells, which are myeloid-derived dendritic cells that co-express CD1a and CD207 (langerin). In addition to LCH cells, these lesions also contain an inflammatory microenvironment of T cells, macrophages, neutrophils, eosinophils, B cells, plasma cells and multinucleated giant cells. Whilst LCH cells are well studied (Figure 1) and important in the pathogenesis of this disease, the inflammatory infiltrate and the corresponding cytokine milieu they produce are central to the resultant organ damage, which is a hallmark of this disease.1 Although LCH is more common in young children, it occurs at all ages, and the resulting mortality/morbidity varies from lethal to mild despite similar pathology of biopsied lesions.
- Publisher
- John Wiley and Sons Inc
- Relation
- Acta Paediatrica, International Journal of Paediatrics Vol. 110, no. 11 (2021), p. 2888-2891
- Rights
- All metadata describing materials held in, or linked to, the repository is freely available under a CC0 licence
- Rights
- https://creativecommons.org/licenses/by-nc/4.0/
- Rights
- Copyright © 2021 The Authors
- Rights
- Open Access
- Subject
- 1114 Paediatrics and Reproductive Medicine
- Full Text
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