What causes Langerhans cell histiocytosis (LCH)?

Updated: Jun 12, 2020
  • Author: Christopher R Shea, MD; Chief Editor: William D James, MD  more...
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The etiology of Langerhans cell histiocytosis (LCH) remains unknown.

Langerhans cell proliferation may be induced by a viral infection, a defect in intercellular communication (T cell and macrophage interaction), and/or a cytokine-driven process mediated by tumor necrosis factor, IL-11, and leukemia inhibitory factor. [22, 23, 24] Specifically, human herpesvirus 6 (HHV-6) has been proposed to contribute to the initiation and/or modulation of persistent LCH. However, other studies have not shown a correlation between HHV-6 and LCH, and their reported associations may represent coincidental findings. [25, 26]

Cigarette smoking may play a role as a chronic irritant in the development of eosinophilic granuloma of the lung. In addition, studies have shown that over 90% of pulmonary LCH were in smokers, with cigarette smoking driving the pathogenesis of pulmonary LCH in mouse models of BRAF V600E mutated mice. [27]

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