Disease Information

Leukemias / Lymphomas: Langerhans Cell Histiocytosis

Alternate Names: LCH, eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease

What is Langerhans cell histiocytosis?

Langerhans cell histiocytosis (LCH) is a disorder in which the body makes too many dendritic cells. These cells play a role in the body’s immune system.

Langerhans cells are a form of dendritic cell, or histiocyte, which is mostly found in the skin, lungs, stomach and intestines. In LCH, these cells build up in various tissues and cause damage.

LCH is not a cancer, but it shares similarities to some cancers and often requires treatment with chemotherapy.

LCH most commonly affects the skin and bones, but it can involve any organ in the body including lymph nodes, lungs, liver, spleen, bone marrow or brain.

Low-risk (less extensive) LCH involves either:

High-risk (more extensive) LCH:

The exact cause of LCH in children is unknown. Past research suggested there may be triggers, such as infections or factors in the environment that can make a child more likely to develop the disease. Newer research shows that LCH arises when mutations (changes) develop in genes that control how dendritic cells behave.

How common is Langerhans cell histiocytosis?

What are the symptoms of Langerhans cell histiocytosis?

Symptoms of Langerhans cell histiocytosis vary widely depending on the body system, organs and tissues affected. Some common symptoms include the following:

How is Langerhans cell histiocytosis treated?

Some children with low-risk LCH—for example, affecting only their skin or a single bone—will improve without therapy. If just a single bone is affected, surgery is usually the first step. The surgery is used to confirm the diagnosis and remove the dendritic-cell growth. Doctors watch the child carefully afterward to check for a possible return of LCH.

For LCH that involves more than one area of the body, the best treatment is chemotherapy (chemo) that lasts about a year. High-risk LCH often requires stronger chemo to cure the disease.

What are the survival rates for Langerhans cell histiocytosis?

Why choose St. Jude for your child’s Langerhans cell histiocytosis treatment?

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