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Langerhans Cell Histiocytosis Treatment

General Information About Langerhans Cell Histiocytosis (LCH)

Langerhans cell histiocytosis is a disease that can damage tissue or cause lesions to form in one or more places in the body.

Langerhans cell histiocytosis (LCH) is a rare disease that begins in LCH cells (a type of dendritic cell which fights infection). Sometimes there are changes in LCH cells as they form. These changes may cause the LCH cells to grow and multiply quickly. This causes the LCH cells to build up in certain parts of the body, where they can damage tissue or form lesions.

LCH is not a disease of Langerhans cells that normally form in skin.

Scientists do not agree on whether LCH is a type of cancer or is a condition caused by a change in the immune system. LCH is mainly treated with anticancer drugs. Some of these drugs are also used to treat immune system conditions.

LCH may occur at any age, but is most common in young children. Treatment of LCH in childhood is different from treatment of LCH in adults. The treatments for LCH in children and adults are described in separate sections of this summary.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood Langerhans cell histiocytosis. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Family history and having a parent who was exposed to certain chemicals may increase the risk of developing LCH.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get the disease; not having risk factors doesn't mean that you will not get the disease. Talk to your doctor if you think you may be at risk. Risk factors for LCH may include the following:

The cause of LCH is not known.

The signs and symptoms of LCH depend on where it occurs in the body.

These and other symptoms may be caused by LCH. Other conditions may cause the same symptoms. Talk to your doctor if any of the following problems occur:

Skin

In infants, signs and symptoms of LCH may include:

In children and adults, signs and symptoms of LCH may include:

Mouth

Signs and symptoms of LCH may include:

Bone

Signs and symptoms of LCH may include:

Lymph nodes and thymus

Signs and symptoms of LCH may include:

Endocrine system (including the pituitary gland)

Signs and symptoms of LCH may include:

Thyroid

Signs and symptoms of LCH may include:

Central nervous system

Signs and symptoms of LCH may include:

Liver and spleen

Signs and symptoms of LCH may include:

Lung

Signs and symptoms of LCH may include:

Bone marrow

Signs and symptoms of LCH may include:

Tests that examine the organs and body systems where LCH may occur are used to detect (find) and diagnose LCH.

The following tests and procedures may be used to detect (find) and diagnose LCH or conditions caused by LCH:

Certain factors affect prognosis (chance of recovery) and treatment options.

LCH in organs such as the skin, bones, lymph nodes, or pituitary gland usually gets better with treatment and is called "low- risk". LCH in the spleen, liver, bone marrow, or lung is harder to treat and is called "high-risk".

The prognosis (chance of recovery) and treatment options depend on the following:

In infants up to one year of age, LCH may go away without treatment.