What Is Childhood Langerhans Cell Histiocytosis?
Langerhans cell histiocytosis (LCH) is a rare disorder that occurs when a child has too many of a certain type of cell called Langerhans cells. These cells normally reside in the skin and help fight infection and destroy foreign substances in the body. In LCH, extra Langerhans cells spread through the blood and build up in certain parts of the body, where they can damage tissue or form tumors.
- In LCH, Langerhans cells typically accumulate in bones but can also accumulate in the skin, lymph nodes, liver, spleen, lung, or brain.
- Scientists do not agree on whether LCH is a type of cancer or a condition caused by a change in the immune system. However, recent studies by scientists at Dana-Farber Cancer Institute have demonstrated a mutant oncogene, BRAF V600E, in more than half of LCH biopsy samples. This suggests that LCH is a type of neoplastic disease (a disease grouping that includes benign, pre-cancerous and cancerous growths).
- Langerhans cell histiocytosis may occur at any age, but is most common in young children under 10.
- LCH is classified as either unifocal (one site) or multifocal (multiple sites).
- LCH is rare, occurring in just five per million people in the U.S.
Langerhans Cell Histiocytosis Symptoms
Symptoms of LCH often depend on where in the body the disease is concentrated. The most common symptoms include:
- Skin rash
- Tenderness or pain originating from a bone
- Loose or lost teeth
- Multiple ear infections
- Eyelid swelling or other vision problems
- Excessive thirst and urination
- Fever and night sweats
- Failure to gain weight
It is important to understand that some symptoms of LCH may resemble those of other more common medical problems. These are general symptoms of the disease and often dependent on where in the body the disease is occurring, but do not include all possible symptoms. Because many of these symptoms can also point to other conditions, it's important to have your child evaluated by a qualified medical professional right away for an accurate diagnosis and prompt treatment.
Langerhans Cell Histiocytosis Causes
Doctors aren't sure what causes LCH. There has been no evidence to suggest that it is caused by anything that can be prevented, and there is nothing that you or your child did or did not do that caused the disease to develop.
How is Langerhans cell histiocytosis diagnosed?
Tests that examine the organs and body systems where LCH may occur are used to detect and diagnose LCH.
Langerhans cell histiocytosis diagnostic tests typically include:
- X-ray – X-rays are often the first diagnostic study, and often give your doctor information regarding the need for further testing.
- Magnetic resonance imaging (MRI) – This test produces detailed images of organs and other structures in your child's body.
- PET-CT scan – This imaging technique combines positron emission tomography (PET) and computerized tomography (CT). A PET/CT scan can provide information about both the structure and function of cells and tissues in the body during one imaging session.
- Tissue biopsy – Sample tissue from the affected areas of the body is needed to determine how much of your child's body is affected by LCH.
There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best possible treatment options.
What are Langerhans cell histiocytosis treatment options?
LCH treatment varies widely. In fact, in some children, no treatment at all is necessary – the disease will go away on its own.
Langerhans cell histiocytosis treatment may include:
- Surgery. Surgery is required initially to obtain a biopsy, and sometimes all of the LCH cells will be removed during that procedure. In most cases, however, further surgery will be needed later to remove all of the LCH cells.
- Medication, including steroids (prednisone), nonsteroidal anti-inflammatories (indomethacin), which can lessen bone pain
- Chemotherapy, a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of chemotherapy drugs work in different ways. Your child may receive chemotherapy orally (as a pill to swallow), subcutaneously (under the skin), or intravenously (IV – as a direct injection into the bloodstream).
- Radiation therapy, which uses high-energy rays (radiation) from a specialized machine to damage or kill abnormal cells and shrink tumors, is very rarely used for LCH therapy. However, in rare situations, small doses of this treatment may be used to help stop the growth of Langerhans cells in specific areas of the body.
Of course, your child's team of doctors will help determine the best approach for your child's unique situation, based on a number of factors including:
- 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 doctors expect the disease to progress
- Your opinion and preferences
Langerhans Cell Histiocytosis (LCH) Treatment at Dana-Farber/Boston Children's
Children and teens with LCH are treated at the Dana-Farber/Boston Children's Histiocytosis Program, one of the top centers worldwide focused on these rare conditions.
At the core of the treatment team are hematologist/oncologists who specialize in treating children with histiocytosis. Because many organ and body systems may be involved with the disease, a patient's treatment team may also include dermatologists, pulmonologists, endocrinologists, gastroenterologists, neurologists, dentists, orthopedic surgeons, neurosurgeons, radiologists, and radiation oncologists. All senior medical staff participate in clinical research activities, so our patients have access to the very best and up-to-date treatments available.