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Langerhans Cell Histiocytosis in Children

Langerhans cell histiocytosis (histiocytosis X) 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.

What is Langerhans cell histiocytosis in children?

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.

Anatomy of a bone, showing blood cells
Everyone has Langerhans cells, which are a form of white cell usually found in the lymph nodes, spleen, skin, liver, lungs, and bone marrow. (Click to Enlarge)

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

The symptoms of Langerhans cell histiocytosis depend on where in the body the Langerhans cells build up. 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. But active treatment is delayed until there is a change in the condition.

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.

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.

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. This includes delayed growth.

  • Anemia

  • Lung failure

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.

Online Medical Reviewer: L Renee Watson MSN RN
Online Medical Reviewer: Richard LoCicero MD
Online Medical Reviewer: Ronald Karlin MD
Date Last Reviewed: 11/1/2018
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