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:
- Skin only;
- Bone only, either a single bone or multiple bones; or
- Multiple organs but not the liver, bone marrow or spleen.
High-risk (more extensive) LCH:
- involves multiple organs including the liver, bone marrow or spleen; and
- is more common in children younger than 2 years old.
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?
- About two to three children out of every million develop Langerhans cell histiocytosis each year.
- It can be found in children at birth and up through the teen years.
- It is most often found in children ages 1 to 3.
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:
- Skin rash, commonly including the scalp
- Bone pain
- Distended abdomen (enlarged belly)
- Increased thirst
- Increased urine output
- Cough or rapid breathing
- Weight loss
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.
- Chemotherapy (“chemo”) — uses powerful medicines to kill cells or stop them from growing (dividing) and making more cells.
- Chemo may be injected into the bloodstream, so that it can travel throughout the body.
- Some chemo may be given by mouth.
- Combination therapy uses more than one type of chemo at a time.
What are the survival rates for Langerhans cell histiocytosis?
- About 99 percent of children with low-risk LCH survive.
- The survival rate for high-risk LCH is about 80 percent.
Why choose St. Jude for your child’s Langerhans cell histiocytosis treatment?
- The nurse-to-patient ratio at St. Jude is unmatched—averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
- St. Jude researchers are looking for ways to improve the prognosis of patients whose organs are at risk and of patients who do not respond to initial therapy.
- Your child will have access to a close-knit team of specialists that includes: surgeons; doctors and nurses who treat this cancer; doctors who specialize in radiation therapy, in hormones (endocrinologists), and in diagnosis (pathologists); experts in diagnostic imaging and nuclear medicine; dietitians; speech therapists; rehabilitation specialists (physical therapists, occupational therapists); child life specialists; psychologists; Quality of Life team members; experts who help manage and minimize the long-term or late effects of treatment; researchers; scientists; and many others.
- The medical team works closely with laboratory researchers to bring new treatments from the laboratory to the clinic.
- New chemo drugs are being studied to see if they are effective for treating LCH.
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