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. 2015 Feb 22;13:13. doi: 10.1186/s12964-015-0092-z

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© Murakami et al.; licensee BioMed Central. 2015

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Proposed relationship between MCPyV and mutated precursor LCs in LCH-risk organ (RO) (−). Mutated LCH precursor cells (mutant LC precursors) in peripheral tissues recognize MCPyV and induce LCH-RO (−). LCH lesion is consisted by accumulation and prolonged survival of LCH cells with T cells, eosinophils, macrophages, etc. In LCH lesions the prominent sources of cytokine storm are T cells and LCH cells. LCH cells produce pro-inflammatory cytokines such as IL-1, anti-inflammatory cytokines such as IL-10, and growth factors. After accumulation and prolonged survival of LCH cells, LCH lesions diminish and spontaneous healing may be observed.