Histiocytic disorders: insights into novel biology and implications for therapy of Langerhans cell histiocytosis and Erdheim-Chester disease.

Abstract excerpt

Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are caused by mutations of the MAPK pathway, most often BRAFV600E, in myeloid dendritic cells that lead to some overlapping and other unique presentations of the two diseases. LCH occurs in both children and adults, but ECD is primarily found in the latter. The challenges in diagnosing these conditions relates to the rarity of the conditions...

Topics

• Adult
• Disease Management
• Erdheim-Chester Disease
• Female
• Histiocytosis, Langerhans-Cell
• Humans
• Male
• Middle Aged
• Mutation
• Protein Kinase Inhibitors
• Proto-Oncogene Proteins B-raf