What is Langerhans cell histiocytosis?

Langerhans cell histiocytosis (also called histiocytosis X) is a rare disorder that primarily affects children. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. Histiocyte cells are a form of white blood cells that help the immune system destroy foreign materials and fight infection. The extra immune cells produced by this condition may form tumors, which can affect parts of the body like the bones and possibly spread to other areas.

Langerhans cell histiocytosis historically was thought of as a cancer-like condition, but more recently researchers have begun to consider it an autoimmune phenomenon in which immune cells begin to overproduce and attack the body instead of fighting infection. In most cases it is not known why the disorder appears, although there may be a genetic link. However, it is not contagious; people with Langerhans cell histiocytosis cannot spread the disorder to others.

Most cases affect children between 1 and 15 years old, with the majority of new cases in children between 5 and 10. About 1 in 200,000 people get Langerhans cell histiocytosis each year.

What are the signs and symptoms of Langerhans cell histiocytosis?

In children, Langerhans cell histiocytosis may involve only one part of the body or many different sites.

Signs and Symptoms of Langerhans Cell Histiocytosis

  • Site:  Skin
    • Possible symptoms: General rash, dandruff
  • Site: Bone
    • Possible symptoms: Lesions, bone pain, limping
  • Site: Lung, liver, spleen
    • Possible symptoms: Dysfunction
  • Site: Teeth and gums
    • Possible symptoms: Loose or lost teeth, swollen gums
  • Site: Ear
    • Possible symptoms: Chronic infections or discharge
  • Site: Eyes
    • Possible symptoms: Vision problems or bulging eyes
  • Site: Central nervous system
    • Possible symptoms: Headache, dizziness, vomiting, excessive thirst, frequent urination
  • Site: General symptoms
    • Possible symptoms: Fever, weakness, fatigue, irritability, swollen lymph glands, failure to gain weight

Not all patients will have every one of these symptoms. And because these symptoms could be caused by another condition not related to Langerhans cell histiocytosis, it is important to seek proper medical attention to receive an accurate diagnosis.

How is Langerhans cell histiocytosis diagnosed?

Your child's doctor may order special x-rays, a CT scan, or blood tests. The extra immune cells produced by this condition may form tumors, which can affect parts of the body like the bones. These tumors produce a punched-out appearance on a bone x-ray. If this punched-out appearance is seen on an x-ray, your child's doctor should refer you to a pediatric hematologist/oncologist. This doctor will coordinate the care and treatment of your child. To assist in determining the extent of disease and appropriate treatment, a surgeon will need to remove a small sample from the skin, bone marrow, or other tissue. This is called a biopsy. The tissue will be looked at under the microscope to see if there is an abundance of Langerhans cells.

How is Langerhans cell histiocytosis treated?

Your child's chance of recovery (prognosis) and choice of treatment will depend on the stage of your child's disease (whether it is just in the bones, called localized disease, or has spread to other places, called disseminated disease). It will also depend on how the cells look under a microscope, your child's general state of health, and his or her age.

This condition is treated with steroids, which suppress immune function. Depending of the extent of disease, children may need further treatment including chemotherapy drugs, small doses of radiation therapy, or surgery.

Cleveland Clinic Children's

Here at Cleveland Clinic Children's, we specialize in the care of children and young adults with blood disorders and cancer. We use a team approach that cares for all members of the family, recognizing that this is a very difficult time for all of them. Our goal is to have our patients get through therapy with as normal a life as possible, and to emerge from therapy knowledgeable about how to maintain their health.

Should you have more questions about the child or young adult in your life with a blood disorder or cancer, please contact the Pediatric Hematology/Oncology Department @ 216.444.3562.

We wish you and your child well.

For more information

The Histiocytosis Association of America provides support and education for families dealing with Langerhans cell histiocytosis. This organization is an international group of parents, physicians, and friends in search of a cure. It provides public and professional education, patient and family support, and stimulation and support of research. For more information, call 856.589.6606 or go to Histio.org.

*Source: National Institutes of Health, Histiocytosis Association of America*

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