title: Langerhans Cell Histiocytosis and Other Histiocytic Diseases of the Lung.
abstract: Several histiocytic disorders may affect the thoracic cavity. Pulmonary Langerhans cell histiocytosis (LCH) is the histiocytic disorder that most frequently manifests with thoracic involvement in adults and is associated with cigarette smoking. The histiocytic disorders follow variable clinical courses ranging from benign disease to life-threatening aggressive disorders. Although the pathogenesis is not fully understood, it is now apparent that some of these disorders are associated with activating mutations in cell proliferative/regulatory pathways. Management of these rare disorders must be individualized. Pharmacologic treatment may include the use of chemotherapeutic agents. Smoking cessation is imperative in the management of pulmonary LCH.
authors: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors: Erin
authors: E
authors: DeMartino
authors: Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors: Ronald S
authors: RS
authors: Go
authors: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA; Department of Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA; Department of Physiology and Biomedical Engineering, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Electronic address: vassallo.robert@mayo.edu.
authors: Robert
authors: R
authors: Vassallo
mesh_terms: D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy; D015618:Histiocytosis, Sinus / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D009154:Mutation; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D012907:Smoking; D016540:Smoking Cessation; D014057:Tomography, X-Ray Computed
chemical_list: C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf
keywords: BRAF; Cigarette smoking; Histiocyte; Interstitial; Langerhans cell; Lung; Macrophage