Different working groups have reviewed available international and national guidelines and recommendations for diagnosis, treatment and follow-up of LCH. A synthesis of this information was generated and published after approval of many involved specialists and is available from the EURO-Histio-Net website linked in the following LCH pages.
· Langerhans Cell Histiocytosis (LCH) – Children
Guidelines for diagnosis, clinical work-up and treatment during childhood; Version 1.1, March 2011 http://www.eurohistio.net/e1623/e1554/e1856/index_eng.html
The Guidelines include:
· 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.
o Biopsy and Histological Examination
o Diagnostic Criteria
· 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:
o Recommended Evaluation in Every Case
o Specific Clinical Scenarios and Recommended Additional Testing
o Definition of Organ Involvement
o Head MRI in LCH Patients
· 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.
o Management Algorithms
o Local Therapy or Careful Observation
o Systemic Therapy
o Treatment Options in Case of Reactivation
· 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
o Monitoring for Permanent Consequences
o Follow-up – Required Tests and Frequency
o Malignancies Associated with Langerhans Cell Histiocytosis
Our thanks to all of our Histio Partners for their input to these pages.