What is Langerhans cell histiocytosis in children?

Langerhans cell histiocytosis (LCH) is a rare disorder. It occurs when there are very high levels of a type of immune cell called a Langerhans cell. In the past, this disorder was thought to be a type of cancer or a condition similar to cancer. Researchers are now finding that it is more likely tied to an autoimmune response. It happens when the body's immune system attacks itself by mistake.

Langerhans cell histiocytosis causes damage to tissues all over the body. The extra Langerhans cells are a type of white blood cell. They flow all over the body. They build up in areas such as the skin, bones, bone marrow, lymph nodes, mouth, spleen, liver, lungs, pituitary gland, and central nervous system. This creates tumors.

What causes Langerhans cell histiocytosis in a child?

This disorder is likely an autoimmune response. It happens when the body's immune system attacks itself by mistake. There are certain gene mutations that control how histiocytes function and cause Langerhans cell histiocytosis. Experts don't think it's inherited. It's not contagious.

What are the symptoms of Langerhans cell histiocytosis in a child?

Symptoms depend on where in the body the Langerhans cells build up. The disorder can occur at any age. These are possible symptoms:

• Pain in the belly or in the bones

• Poor growth (failure to thrive)

• Irritability

• Yellowing of the skin and eyes (jaundice)

• Urinating often and having constant thirst. This is caused by diabetes insipidus.

• Dark red or brown sores (lesions) on the skin, especially on the face, scalp, back, and groin

• Bulging eyes

• Constant drainage from the ear

• Headaches

• Flaking skin on the scalp similar to cradle cap

• Thyroid problems

• Problems with balance, memory, or behavior

• Swollen gums, mouth sores, and tooth loss

How is Langerhans cell histiocytosis diagnosed in a child?

A healthcare provider may diagnose Langerhans cell histiocytosis through several things. These include:

• Physical exam

• Discussion of symptoms or illnesses

• Nervous system exam. This is to assess the spinal cord, brain, and nerves, and mental functioning.

Your child may also need to have a number of lab tests. These include blood tests, urine tests, and a biopsy of the bone marrow. Your child may also need imaging tests such as a bone scan, CT scan, MRI, ultrasound, or PET scan.

How is Langerhans cell histiocytosis treated in a child?

Treatment typically includes methods to treat cancer:

• Chemotherapy

• Radiation therapy

• Surgery to remove abnormal tissues

• Corticosteroid medicines

• Photodynamic therapy

Some parents of children with Langerhans cell histiocytosis may choose watchful waiting. This means the healthcare provider closely watches symptoms and progress of the condition. Active treatment may be delayed until there is a change in the condition.

What are possible complications of Langerhans cell histiocytosis in a child?

Langerhans cell histiocytosis can cause damage to tissues and organs all over the body if it's not treated. One example is pulmonary histiocytosis. This condition damages the lungs. Damage to the body can be so severe that the condition becomes fatal. Other complications may include:

• Pituitary gland problems

• Growth problems, including delayed growth

• Anemia

• Lung failure

Can Langerhans cell histiocytosis be prevented in a child?

Experts don't know what causes Langerhans cell histiocytosis. So there is no known way to prevent the condition. Having a family history of thyroid disease may increase the risk.

Some other factors can increase your child's risk of developing the condition. Limit your exposure to certain chemicals, including benzene.

When should I call my child's healthcare provider?

Parents should watch children carefully and look for any warning signs of the disease. Bring up any concerns, growth problems, or abnormal symptoms with your child's healthcare provider.

Key points about Langerhans cell histiocytosis in children

• Langerhans cell histiocytosis is a rare disorder that damages tissues all over the body. It occurs when your child has very high levels of a type of immune cell (Langerhans cell).

• The extra cells travel all over the body. The cells build up and create tumors. These tumors can be in areas such as the skin, bones, bone marrow, lymph nodes, mouth, spleen, liver, and lungs.

• It is likely tied to an autoimmune response. It happens when the body's immune system attacks itself by mistake.

• Symptoms depend on where in the body the Langerhans cells build up. Possible symptoms include belly or bone pain, irritability, poor growth, bulging eyes, and brown or red skin sores.

• Experts don't know what causes it. Having a family history of thyroid disease may increase the risk.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.

• Before your visit, write down questions you want answered.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.

• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are and when they should be reported.

• Ask if your child’s condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if your child does not take the medicine or have the test or procedure.

• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your child’s provider after office hours and on weekends and holidays. This is important if your child becomes ill and you have questions or need advice.