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300 Longwood Avenue
Boston, MA 02115
(617) 355-6000
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My Child Has:
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Langerhans Cell Histiocytosis
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Langerhans cell histiocytosis is a rare disorder that occurs when there are too many of a type of white blood cell called a Langerhans cell (named for a German scientist). These cells normally reside in the skin and help fight infections and destroy certain foreign substances in the body. In LCH, these cells accumulate on bones and other parts of the body, particularly the head and neck, causing a wide range of problems. LCH can also be found in the ribs, sternum, long bones of the arms and legs, vertebra of the spine, and the pelvis. Although LCH can occur in people of all ages, a majority of cases occur in children under 10 years old.
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The cause of Langerhans cell histocytosis is not entirely understood. Researchers are studying viral infections or environmental factors that could lead to this disorder.
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LCH can result in symptoms in one or several parts of the body. Common symptoms include:
- skin rash
- tenderness or pain originating from a bone
- loose or lost teeth
- swollen gums
- multiple ear infections
- eyelid swelling and other vision problems
- excessive thirst and urination
- fever and night sweats
- weakness and failure to gain weight
The symptoms of LCH vary widely and may resemble other medical conditions. Always consult your child's physician for a diagnosis.
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In addition to a complete medical history and physical examination, diagnostic procedures for LCH may include:
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- biopsy - a simple surgical procedure by which a physician removes a small sample of affected body tissue so that
- multiple imaging studies of the areas of the body affected, such as:
- x-rays - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
- bone scans - a nuclear imaging method to detect bone diseases and tumors and to determine the cause of bone pain or inflammation.
- magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
- computerized tomography scan (also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
- complete blood count (CBC) - a measurement of size, number
- other blood tests (including blood chemistries)
- liver or bone marrow biopsy - sample tissue from these areas of the body can be helpful in determine how much of the body is affected by LCH
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Specific treatment for LCH will be determined by your child's physician based on:
- your child's age, overall health, and medical history
- the extent of the disease
- your child's tolerance for certain medications, procedures, or therapies
- how your child's physician expects the disease to progress
- your opinion or preference
Treatment for LCH also varies widely. In some cases, the disease will regress without treatment. For others, treatment may include:
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- medications - steroids, hormones and other drugs
- surgery - surgical removal of growths of LCH cells
- radiation therapy - small doses of this treatment, usually used against cancer, can help stop the growth of Langerhans cells in specific areas of the body. Radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill abnormal cells.
- chemotherapy - small doses of this treatment, usually used against cancer, can help stop the growth of Langerhans cells throughout the body. Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill or slow the growth of targeted cells. Chemotherapy can be given:
- as an injection into the muscle or fat tissue
- intravenously (directly to the bloodstream; also called IV)
- intrathecally - chemotherapy given directly into the spinal column with a needle
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While chemotherapy can be quite effective in treating certain cancers, the agents do not completely differentiate normal healthy cells from abnormal cells. Because of this, there can be adverse side effects during treatment.
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Most patients with LCH will survive the disease, although some may develop long-term chronic health problems. If the disease occurs in an infant, the chances of severe disease and fatality are much greater. The response to this disease and its treatments vary widely, so speak with your child's physician about your individual situation.
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The information on this website should not be taken as medical advice,
which can only be given to you by your personal health care professional. |
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Copyright © Children's Hospital Boston. All rights reserved. |
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