Langerhans Cell Histiocytosis - Adults
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.
o Stratification / Clinical classification
o Risk organs and special sites
o Endocrinologic dysfunction
o Dermatological involvement
o Gastrointestinal involvement
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.