title: Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net.
abstract: Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Adult LCH patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of other systems, resulting in patients being underdiagnosed and/or incompletely staged. Furthermore they may be treated with pediatric-based therapies which are less effective and sometimes more toxic for adults. The published literature on adult LCH cases lacks a comprehensive discussion on the differences between pediatric and adult patients and there are no recommendations for evaluation and comparative therapies. In order to fill this void, a number of experts in this field cooperated to develop the first recommendations for management of adult patients with LCH. Key questions were selected according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Based on the available literature up to December 2012, recommendations were established, drafts were commented by the entire group, and redrafted by the executive editor. The quality of evidence of the recommendations is predominantly attributed to the level of expert opinion. Final agreement was by consensus.
authors.affiliation: Department of Medicine I, Center of Hematology an Stem Cell Transplantation, Hemostasis and Medical Oncology Internal Medicine I, Elisabethinen Hospital, Fadinger Str, 1 4010, Linz, Austria. michael.girschikofsky@elisabethinen.or.at
authors.forename: Michael
authors.initials: M
authors.lastname: Girschikofsky
authors.forename: Maurizio
authors.initials: M
authors.lastname: Arico
authors.forename: Diego
authors.initials: D
authors.lastname: Castillo
authors.forename: Anthony
authors.initials: A
authors.lastname: Chu
authors.forename: Claus
authors.initials: C
authors.lastname: Doberauer
authors.forename: Joachim
authors.initials: J
authors.lastname: Fichter
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.forename: Gregory A
authors.initials: GA
authors.lastname: Kaltsas
authors.forename: Polyzois
authors.initials: P
authors.lastname: Makras
authors.forename: Angelo V
authors.initials: AV
authors.lastname: Marzano
authors.forename: Mathilde
authors.initials: M
authors.lastname: de Menthon
authors.forename: Oliver
authors.initials: O
authors.lastname: Micke
authors.forename: Emanuela
authors.initials: E
authors.lastname: Passoni
authors.forename: Heinrich M
authors.initials: HM
authors.lastname: Seegenschmiedt
authors.forename: Abdellatif
authors.initials: A
authors.lastname: Tazi
authors.forename: Kenneth L
authors.initials: KL
authors.lastname: McClain
mesh_terms: D000328:Adult; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy*; D006801:Humans; D008297:Male; D017410:Practice Guidelines as Topic