Langerhans Cell Histiocytosis (LCH)
Author(s): Euro Histio Net Work Group for LCH Guidelines, Created: 2011/03/09, last update: 2013/03/24
Guidelines for diagnosis, clinical work-up and treatment during childhood; Version 1.1, March 2011
Riccardo Haupt (Leader and Editor)1, Itziar Astigarraga2, Eva Schaefer3, Jean Donadieu3, Maarten Egeler4, Gritta Janka5, Rima Jubran6, Dragan Micic7, Milen Minkov8, Vasanta Nanduri9, Carlos Rodriguez-Galindo10, Stefaan Van Gool11, Johannes Visser12, Sheila Weitzman13
1 Istituto G. Gaslini, Genova, Italy; 2 Hospital de Cruces, Barakaldo, Spain; 3 Centre de réference des histiocytoses, Hopital Trousseau, Assistance Publique – Hopitaux de Paris, France; 4 Universiteit Leiden, The Netherlands; 5 Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany; 6 Children's Hospital of Los Angeles, California, USA; 7 Institut za zdravstvenu zaštitu majke i deteta Srbije “Dr Vukan Čupić”, Novi Beograd, Serbia; 8 Kinderkrebsforschung, St. Anna Kinderspital, Wien, Austria; 9 Watford General Hospital, Watford, United Kingdom; 10 Dana Farber Cancer Institute and Children’s Hospital Boston, Massachusetts, USA; 11 Universitair ziekenhuis (UZ) Leuven, Belgium; 12 University Hospitals of Leicester, LRI Children’s Hospital, Leicester, United Kingdom; 13 Hospital for Sick Children, Toronto, Ontario, Canada
Table of Contents
- Methods and Process of development
- Purpose and Restrictions
- General Considerations
Definitive diagnosis of LCH requires classical histopathology confirmed by demonstration of CD1a or Langerin (CD207) or by the ultrastructural presence of Birbeck granules.
- Pretreatment Clinical Evaluation
The following patient evaluation is recommended only for patients with definite diagnosis of LCH and for those with presumptive LCH in whom a biopsy is deferred for the reasons mentioned in the chapter Biopsy / Histology. In all other cases we suggest either consulting the referral center of your country or sharing the histological slides with other reference or specialized centers.
- Clinical Classification
Treatment recommendations are based on site and extension of the disease which is why it is important to distinguish between single system LCH and multisystem LCH.
- Differential Diagnosis
Whenever possible patients should be enrolled in ongoing clinical trials and treated according to the protocol. CHILDREN who are not enrolled in a clinical trial should be treated according to the guidelines presented here. Due to the diversity of clinical course of LCH, even recommendations which are established as standard of care may need to be critically appraised in an individual case. We suggest that you never hesitate to contact LCH experts in case that the clinical course raises questions or doubts. ADULTS should NOT be treated according to these guidelines before prior consultation of an expert.
- Follow-up of LCH patients
General Considerations; Diabetes insipidus; Hormone Deficiencies; Orthopaedic Sequelae; Problems with Ears, Oral Tissue and Jaw; Neurological Sequelae; Damage of Lung and Liver
- Reference List for the guidelines for diagnosis, clinical work-up and treatment during childhood