previously known as Histiocytosis X
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) – Adults
Recommendations for diagnosis, clinical work-up and treatment of adults; Version 1, August 2013
The Guidelines include:
· Background, Process of Development and Restrictions
There are no universally accepted international guidelines available for the diagnosis and treatment of adult LCH patients.
· General consideration
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
o History and physical examination
o Laboratory and radiographic evaluation
o Further investigations for specific clinical scenarios
· Organ involvement
After the diagnosis of LCH has been made, involvement of other organs should be evaluated and defined according to the clinical, biological or radiological criteria.
Treatment recommendations are based on site and extension of the disease.
o Management algorithm
o Careful observation, local or “mild systemic” therapy
o Systemic therapy
o Treatment options in case of reactivation
o Treatment and hormone replacement of endocrinopathies
o Central nervous system involvement
Although the lungs may be affected simultaneously with other organs an isolated pulmonary LCH is observed more frequently and represents a special form of adult LCH.
o Epidemiology and clinical features
o Treatment and prognosis
LCH may reactivate and lead to chronic local symptoms or induce organ dysfunction. Rarely LCH is associated with malignant tumors. Therefore, follow-up investigations of disease and monitoring of functional impairments are necessary.
Our thanks to all of our Histio Partners for their input to these pages.