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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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Overview



What is Langerhans cell histiocytosis (LCH)?

What are the signs and symptoms of Langerhans cell histiocytosis?

What causes Langerhans cell histiocytosis?

Is Langerhans cell histiocytosis inherited?

How is Langerhans cell histiocytosis diagnosed?

How might Langerhans cell histiocytosis be treated?


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 causes Langerhans cell histiocytosis?

The cause of Langerhans cell histiocytosis is unknown.[3][4] It may be triggered by an unusual reaction of the immune system to something commonly found in the environment. It is not considered to be an infection or cancer. It is not known to be hereditary or communicable.[3]
Last updated: 12/2/2013

Is Langerhans cell histiocytosis inherited?

Although Langerhans cell histiocytosis is generally considered a sporadic, non-hereditary condition, it has reportedly affected more than one individual in a family in a very limited number of cases (particularly identical twins).[5][6]
Last updated: 12/2/2013

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][7]

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

References
  1. LCH in Children. Histiocytosis Association of America. https://www.histio.org/page.aspx?pid=379. Accessed 5/7/2012.
  2. Grifo AH. Langerhans Cell Histiocytosis in Children. Association of Pediatric Hematology/Oncology Nurses. 2009;
  3. LCH in Children. Histiocytosis Association of America. 2007; http://www.histio.org/site/c.kiKTL4PQLvF/b.1764433/k.8BCD/LCH_in_Children.htm. Accessed 2/18/2010.
  4. Shea CR, Boos MD. Langerhans Cell Histiocytosis. eMedicine. 2009; http://emedicine.medscape.com/article/1100579-overview. Accessed 2/18/2010.
  5. Langerhans Cell Histiocytosis. Online Mendelian Inheritance in Man (OMIM). 2009; http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=604856. Accessed 2/18/2010.
  6. Arico M, Nichols K, Whitlock JA, Arceci R, Haupt R, Mittler U, Kuhne T, Lombardi A, Ishii E, Egeler RM, Danesino C. Br J Haematol. 1999; http://www.ncbi.nlm.nih.gov/pubmed/10606898?dopt=Abstract. Accessed 2/18/2010.
  7. Satter EK, High WA. Langerhans Cell Histiocytosis: A Review of the Current Recommendations of the Histiocyte Society. Pediatric Dermatology. 2008;