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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Langerhans cell histiocytosis


Other Names for this Disease
  • Eosinophilic granuloma (formerly)
  • Hand-Schüller-Christian syndrome (formerly)
  • Histiocytosis X
  • LCH
  • Letterer-Siwe disease (formerly)
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Your Question

My child had surgery for Langerhans cell histiocytosis. I believe that I was once told that this condition is not genetic. Is this true?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Langerhans cell histiocytosis (LCH)?

Langerhans cell histiocytosis (LCH) is a disorder that primarily affects children, but is also found in adults of all ages. People with LCH produce too many Langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. In people with LCH, these cells multiply excessively and build up in certain areas of the body, causing tumors called granulomas to form. The symptoms vary among affected individuals, and the cause of LCH is unknown. In most cases, this condition is not life-threatening. Some people do experience life-long problems associated with LCH.[1]
Last updated: 10/18/2013

What causes Langerhans cell histiocytosis?

The cause of Langerhans cell histiocytosis is unknown.[2][3] It may be triggered by an unusual reaction of the immune system to something commonly found in the environment. It is not considered to be an infection or cancer. It is not known to be hereditary or communicable.[2]
Last updated: 12/2/2013

Is Langerhans cell histiocytosis inherited?

Although Langerhans cell histiocytosis is generally considered a sporadic, non-hereditary condition, it has reportedly affected more than one individual in a family in a very limited number of cases (particularly identical twins).[4][5]
Last updated: 12/2/2013

References