Figure 5
From: Interleukin-1 loop model for pathogenesis of Langerhans cell histiocytosis
Proposed relationship between MCPyV and mutated monocytes in LCH-RO (+). Mutated precursor LCH cells (mutant monocytes) in blood vessels recognize MCPyV and induce LCH-RO (+). LCH lesion is consisted by accumulation and prolonged survival of LCH cells with T cells, eosinophils, macrophages, etc., which is not different from that of LCH-RO (−). 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. But circulating monocytes with mutation triggered by MCPyV caused disseminated LCH lesions including RO.