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» Expert Histio Net » For Professionals » Guidelines » LCH adult » Background/Restrictions  · 

Background, Process of Development and Restrictions

Author(s): Euro Histio Net Work Group for LCH Guidelines for adults (see introduction page), Created: 2012/07/26, last update: 2013/11/20

There are no universally accepted international guidelines available for the diagnosis and treatment of adult LCH patients.

Langerhans Cell Histiocytosis (LCH) - Adults
Recommendations for diagnosis, clinical work-up and treatment of adults; Version 1, August 2013

The largest number of patients was published in a pooled retrospective analysis from several national registries [Arico et al 2003].

Based on the available literature up to December 2012 and personal experience the following recommendations were established by an international group of academic clinicians who are recognized experts in the field of histiocytic disorders. Grading of recommendations based on levels of evidence and agreement between experts is listed in table 1.

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 and involvement of a LCH expert should be considered. A map of experts, reference centers and additional information about the disease is available in this webportal and on the website of the the Histiocytosis Association.

Tab. 1. Grade of Recommendation


Level of evidence


Level of agreement between experts


meta-analyses, high quality systematic reviews, or randomized controlled trials


general agreement between all experts


systematic reviews of case control or cohort studies


discussed recommendation, but no formal objections between experts


non-analytic studies: for example, case reports, case series, small retrospective studies


divergence of opinion


expert opinion


[Arico et al 2003] Aricò M, Girschikofsky M, Généreau T, Klersy C, McClain K, Grois N, Emile JF, Lukina E, De Juli E, Danesino C: Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society. European journal of cancer (Oxford, England : 1990) 2003, 39: 2341 [PMID: 14556926]