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Genetic and Rare Diseases Information Center (GARD)

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Langerhans cell histiocytosis


Other Names for this Disease
  • Eosinophilic granuloma (formerly)
  • Hand-Schüller-Christian syndrome (formerly)
  • Histiocytosis X
  • LCH
  • Letterer-Siwe disease (formerly)
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Your Question

My toddler daughter has been diagnosed with Langerhans cell histiocytosis. I'd like to learn as much as possible about the condition.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Langerhans cell histiocytosis (LCH)?

Langerhans cell histiocytosis (LCH) is a disorder that primarily affects children, but is also found in adults of all ages. People with LCH produce too many Langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. In people with LCH, these cells multiply excessively and build up in certain areas of the body, causing tumors called granulomas to form. The symptoms vary among affected individuals, and the cause of LCH is unknown. In most cases, this condition is not life-threatening. Some people do experience life-long problems associated with LCH.[1]
Last updated: 10/18/2013

What are the signs and symptoms of Langerhans cell histiocytosis?

Symptoms of Langerhans cell histiocytosis (LCH) can vary greatly from person to person depending on how much of the body is involved and what part(s) are affected. The disease can affect virtually every organ, including skin, bones, lymph nodes, bone marrow, liver, spleen, lungs, gastrointestinal tract, thymus, central nervous system, and hormone glands. The symptoms may range from localized bone lesions or skin disease to multiple organ involvement and severe dysfunction.[2]

Below are the organs that may be affected as well as the symptoms that might be observed:[2]

  • Skin - Red, scaly papules in areas where opposing skin surfaces touch or rub (e.g. skin folds) are commonly seen in LCH. Infants with the skin presentation on the scalp are often misdiagnosed with cradle cap. The skin symptoms usually improve without treatment.
  • Bone - Lesions that cause bone destruction are common, with the skull, lower limbs, ribs, pelvis, and vertebrae usually being affected. Symptoms may include pain, swelling, limited motion, and inability to bear weight.
  • Lymph node - Lymph node involvement may be limited or associated with a skin or bone lesion or disseminated disease. Although any of the lymph nodes may be affected, the cervical lymph nodes are where the disease commonly occurs. Individuals usually only present with pain of the lymph node affected. If only one lymph node is affected, prognosis is normally good and treatment is unnecessary.
  • Liver - Liver involvement at the time of diagnosis is generally associated with more severe disease. Symptoms may include ascites, jaundice, low levels of protein, and prolonged clotting time.
  • Central nervous system (CNS) and hormone - CNS involvement is rare and may be devastating. The most common result of CNS involvement is the altering of hormonal function, with some individuals developing diabetes insipidus.

More detailed information about the symptoms of LCH can be accessed through the Histiocytosis Association's website.

Last updated: 12/2/2013

What is known about who develops Langerhans cell histiocytosis?

  • More than 50 percent of cases are observed in children under 15 years old, with a peak incidence at 1 to 4 years of age.  However, Langerhans cell histiocytosis can affect people of all ages.[2]
  • There is no ethnic association.[2]
  • The prevalence is slightly higher in males. [2]
Last updated: 5/7/2012

How is Langerhans cell histiocytosis diagnosed?

Testing for Langerhans cell histiocytosis (LCH) may include bronchoscopy with biopsyx-ray, skin biopsy, bone marrow biopsy, complete blood count, and pulmonary function tests. Because LCH is sometimes associated with cancer, CT scans and a biopsy may be done to rule out possible cancer.[2][3]

Additional information about the diagnosis of LCH can be viewed on the Histiocytosis Association's website.
Last updated: 12/2/2013

How might Langerhans cell histiocytosis be treated?

Treatment for Langerhans cell histiocytosis (LCH) depends upon the individual patient; it may differ depending on the type and severity of the condition as well as what part(s) of the body are affected. In some cases, the disease will regress without any treatment at all. In other cases, limited surgery and small doses of radiation therapy or chemotherapy will be needed, depending on the extent of the disease. Treatment is planned after complete evaluation of the patient, with the goal of using as little treatment as possible to keep the disease under control.[1]

Detailed information about the treatment of LCH can be viewed on Medscape Reference's Web site.
Last updated: 12/2/2013

Who might treat Langerhans cell histiocytosis?

Langerhans cell histiocytosis is generally treated by a team composed of health care professionals from different specialties.  However, over the years, cancer treatments have been used in patients with histiocytosis. Therefore, hematologists and oncologists are usually part of the multidisciplinary team. The disease is not cancer. Radiation therapy, if used, is given in much lower doses than that which cancer patients receive.[1]  
Last updated: 5/7/2012

What is the typical prognosis for people with Langerhans cell histiocytosis?

The prognosis (chance of recovery) for people with Langerhans cell histiocytosis can vary greatly from patient to patient, but in the majority of children, the disease resolves itself. Prognosis seems to be dependent mainly on the number of organ systems involved, the severity of organ involvement, and to a lesser rate, the age at which symptoms occur.[2][3]  In general, patients who are young and those with disseminated disease and organ dysfunction tend to have a poorer prognosis.[2][3] Newborns who present only with skin lesions tend to do well. Therefore, the age at presentation is only important when multiple organs are affected. Additionally, individuals who have liver, spleen, lung, or bone marrow involvement usually have a worse prognosis. In a study looking at patients from several centers, it was shown that the best prognostic indicator was the patient's response to chemotherapy during the first six weeks of therapy. Therefore, it has been recommended by some that individuals who do not respond positively within the first six weeks of treatment should be treated more aggressively.[3]   
Last updated: 5/7/2012

How can I learn about research involving Langerhans cell histiocytosis?

You can learn more about ongoing clinical research studies on the ClinicalTrials.gov Web site, a site developed by the U.S. National Institutes of Health, through the National Library of Medicine. Currently, there are several clinical trials identified as enrolling individuals with LCH. To find these trials click here.  You can also search for studies by clicking on ClinicalTrials.gov and using 'Langerhans cell histiocytosis' as your search term. After you click on a study, review its 'eligibility' criteria to determine its appropriateness. Use the study’s contact information to learn more. Check this site often for regular updates.
Last updated: 5/7/2012

Where can I learn more about Langerhans cell histiocytosis?

You can visit the following link to view resources related to Langerhans cell histiocytosis.
http://rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=6858
Last updated: 5/7/2012

References
  • LCH in Children. Histiocytosis Association of America. https://www.histio.org/page.aspx?pid=379. Accessed 5/7/2012.
  • Grifo AH. Langerhans Cell Histiocytosis in Children. Association of Pediatric Hematology/Oncology Nurses. 2009;
  • Satter EK, High WA. Langerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte Society. Pediatric Dermatology. 2008;