Open Access Highly Accessed Review

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

Michael Girschikofsky1*, Maurizio Arico2, Diego Castillo3, Anthony Chu4, Claus Doberauer5, Joachim Fichter6, Julien Haroche7, Gregory A Kaltsas8, Polyzois Makras9, Angelo V Marzano10, Mathilde de Menthon11, Oliver Micke12, Emanuela Passoni10, Heinrich M Seegenschmiedt13, Abdellatif Tazi14 and Kenneth L McClain15

Author Affiliations

1 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

2 Department of Pediatric Hematology Oncology, Azienda Ospedaliero Universitaria A. Meyer, Florence, Italy

3 Departament of Respiratory Medicine, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain

4 Imperial NHS Trust, London, UK

5 Clinic for Internal Medicine, Protestant Clinics, Gelsenkirchen, Germany

6 Paracelsus Klinik, Osnabrück, Germany

7 Service de Medicine Interne, Groupe Hospitalier Pitie-Salpetiere, Paris, France

8 Department of Pathophysiology, University of Athens School of Medicine, Athens, Greece

9 Department of Endocrinology and Diabetes, 251 Hellenic Air Force & VA General Hospital, Athens, Greece

10 U.O. Dermatologia, Fondazione IRCCS Ca´ Granda-Ospedale Maggiore Policlinico, Milano, Italy

11 Department of Internal Medicine, Hospital Saint Louis, Paris, France

12 Department of Radiotherapy and Radiation Oncology, Franziskus Hospital, Bielefeld, Germany

13 Radiation Oncology Center, Hamburg, Germany

14 Pulmonolgy Department, Saint Louis Teaching Hospital, Paris, France

15 Texas Children’s Cancer Center/Hematology Service, Houston, TX, USA

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Orphanet Journal of Rare Diseases 2013, 8:72  doi:10.1186/1750-1172-8-72

Published: 14 May 2013

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.

Keywords:
Langerhans; Adult; Histiocytosis