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Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net

Orphanet journal of rare diseases, 2013-05, Vol.8 (1), p.72-72 [Peer Reviewed Journal]

COPYRIGHT 2013 BioMed Central Ltd. ;COPYRIGHT 2013 BioMed Central Ltd. ;2013 Girschikofsky et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. ;Copyright © 2013 Girschikofsky et al.; licensee BioMed Central Ltd. 2013 Girschikofsky et al.; licensee BioMed Central Ltd. ;ISSN: 1750-1172 ;EISSN: 1750-1172 ;DOI: 10.1186/1750-1172-8-72 ;PMID: 23672541

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Title:
Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net
Author: Girschikofsky, Michael ; Arico, Maurizio ; Castillo, Diego ; Chu, Anthony ; Doberauer, Claus ; Fichter, Joachim ; Haroche, Julien ; Kaltsas, Gregory A ; Makras, Polyzois ; Marzano, Angelo V ; de Menthon, Mathilde ; Micke, Oliver ; Passoni, Emanuela ; Seegenschmiedt, Heinrich M ; Tazi, Abdellatif ; McClain, Kenneth L
Subjects: Adult ; Adults ; Autoimmune diseases ; Care and treatment ; Children ; Development and progression ; Diabetes ; Diagnosis ; Experts ; Female ; Health aspects ; Hematology ; Histiocytosis, Langerhans-Cell - diagnosis ; Histiocytosis, Langerhans-Cell - drug therapy ; Histiocytosis, Langerhans-Cell - therapy ; Humans ; Internal medicine ; Langerhans-cell histiocytosis ; Male ; Medical research ; Medicine ; Medicine, Experimental ; Oncology ; Practice Guidelines as Topic ; Pregnancy ; Review ; Smoking cessation ; Teaching hospitals
Is Part Of: Orphanet journal of rare diseases, 2013-05, Vol.8 (1), p.72-72
Description: 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.
Publisher: England: BioMed Central Ltd
Language: English
Identifier: ISSN: 1750-1172
EISSN: 1750-1172
DOI: 10.1186/1750-1172-8-72
PMID: 23672541
Source: Open Access: DOAJ Directory of Open Access Journals
Directory of Open Access Scholarly Resources (ROAD)
Freely Accessible Journals
Open Access: PubMed Central
SpringerOpen
AUTh Library subscriptions: ProQuest Central
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Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net

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