What is Langerhans cell histiocytosis?

Langerhans cell histiocytosis (LCH) is type of cancer in which the bone marrow produces too many of a type of immune cell called Langerhans cells. These cells collect in groups with other cells in the immune system to cause sores or growths called lesions. These lesions can appear in bones, skin, lymph nodes, and almost any organ of the body.

LCH often affects children between 1 and 15 years old, usually occurring between 5 and 10 years of age. It is most prevalent in people of Caucasian descent, and happens in males twice as often as females.

There are 3 types of LCH, which your health care provider might discuss with you:

• Unifocal, meaning there is only one lesion in one part of the body
• Multifocal unisystem, meaning there are multiple lesions in one part of the body
• Multifocal multisystem, meaning multiple lesions in more than one part of the body

Common symptoms include bone lesions, which may or may not be accompanied by diabetes inspidius, a blood sugar disorder caused when LCH causes damage to the pituitary gland, and, and/or a bulging of the eyes, called exophthalmos.

Our approach to Langerhans cell histiocytosis

Diagnosis of LCH is determined by removing a small amount of tissue and performing a test called a biopsy to look for Langerhans cells. If LCH is suspected, an electron microscope test of tissues may be performed.

The treatment choices we make are guided by how far the disease has spread. A single bone lesion may be removed by surgery or treated with radiation. When lesions have spread throughout the system, chemotherapy or other medications may be required over a period of time.