title: 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%.
authors.affiliation: Department of Pediatric Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue D3-133, Boston, MA 02215, USA.
authors.forename: Chalinee
authors.initials: C
authors.lastname: Monsereenusorn
authors.affiliation: Department of Pediatric Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue D3-133, Boston, MA 02215, USA; Department of Pediatrics, Harvard Medical School, Boston, MA, USA. Electronic address: carlos_rodriguez-galindo@dfci.harvard.edu.
authors.forename: Carlos
authors.initials: C
authors.lastname: Rodriguez-Galindo
mesh_terms: D003131:Combined Modality Therapy; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy*; D006801:Humans; D016896:Treatment Outcome
keywords: BRAF; Chemotherapy; Langerhans cell histiocytosis; Neurodegeneration; Oncogenes