Clinical Characteristics and Treatment of Langerhans Cell Histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is a disease caused by clonal proliferation of CD1a+/CD207+ cells that is characterized by a spectrum of varying degrees of organ involvement and dysfunction. Treatment of LCH is risk adapted; patients with single lesions may respond well to local treatment, whereas patients with multi-system disease and risk-organ involvement require more intensive therapy. Although survival for patients without organ dysfunction is excellent, mortality rates for patients with organ dysfunction may reach 30% to 40%. For patients with low-risk disease, although cure is almost universal, disease reactivation rates are in excess of 30%.

Keywords: BRAF; Chemotherapy; Langerhans cell histiocytosis; Neurodegeneration; Oncogenes.