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  • Title: Systemic Histiocytosis (Langerhans Cell Histiocytosis, Erdheim-Chester Disease, Destombes-Rosai-Dorfman Disease): from Oncogenic Mutations to Inflammatory Disorders.
    Author: Papo M, Cohen-Aubart F, Trefond L, Bauvois A, Amoura Z, Emile JF, Haroche J.
    Journal: Curr Oncol Rep; 2019 May 21; 21(7):62. PubMed ID: 31115724.
    Abstract:
    PURPOSE OF REVIEW: Provide an overview of recent progress in decoding the pathogenesis and treatment of systemic histiocytoses. RECENT FINDINGS: Advances in molecular techniques over the last few years, enabling the identification of several MAPK mutations in lesion histiocytes, have revolutionized our understanding of histiocytosis that led to a revised classification and new treatments. Since the 2010 discovery of the BRAFV600E mutation in 57% of Langerhans cell histiocytosis (LCH) lesions, several other kinase mutations have been found, mostly in the MAPK pathway, and also in other key signaling pathways, in LCH, Erdheim-Chester Disease (ECD) and, less frequently, Destombes-Rosai-Dorfman disease (RDD). Those revolutionary breakthroughs enhanced our understanding of the pathogenesis of histiocytosis and led to trials with targeted therapies that demonstrated notable efficacy.
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