The medical guidelines for childhood LCH have been published in Pediatric Blood & Cancer  (PBC), Wiley Online Library.

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» Expert Histio Net » For Professionals » Guidelines » LCH childhood » General Considerations  · 

General Considerations

Author(s): Euro Histio Net Work Group for LCH Guidelines (see introduction page), Created: 2011/03/09, last update: 2012/01/23

The disease may affect any organ or system of our body, but those more frequently affected are the bone (80% of cases), the skin (33%), and the pituitary (25%). Other organs often involved are the liver, spleen, the hematopoietic system and the lungs (15% each). Finally, the lymph nodes (5-10%) and the central nervous system excluding the pituitary (2-4%) are the other possible localization sites of the disease (see other pages of the web portal).

Clinical manifestations of the disease vary depending on the organ or system affected, ranging from an isolated lesion as in the skin or in the bone or in the lung in adults, to a more severe clinical manifesttation affecting the same tissue in multiple sites, or several organs. Thus, the clinical course may vary from self-limiting and self-healing disease to a rapidly progressive one, that might lead to death. Another important aspect of this disease is that between 30 and 40% of affected patients may develop permanent consequences that in some cases may severely affect quality of life either during the acute illness, or later in life. Examples are neuroendocrine deficits (e.g. diabetes insipidus), orthopedic sequelae (e.g. scoliosis, vertebra plana), chronic lung dysfunction, neurologic or neuropsychological deficits, sclerosing cholangitis, teeth loss, and deafness.

Treatment options vary depending on disease extent and severity at onset, and on the response to front-line treatment. Thus, after diagnosis is confirmed, it is important to have the diagnostic and the clinical work-up performed according to uniform guidelines.