General Information About Langerhans Cell Histiocytosis (LCH)
- Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body.
- Family history or having a parent who was exposed to certain chemicals may increase the risk of LCH.
- The signs and symptoms of LCH depend on where it is in the body.
- Skin and nails
- Lymph nodes and thymus
- Endocrine system
- Central nervous system (CNS)
- Liver and spleen
- Bone marrow
- Tests that examine the organs and body systems where LCH may occur are used to detect (find) and diagnose LCH.
- Certain factors affect prognosis (chance of recovery) and treatment options.
Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body.
Langerhans cell histiocytosis
(LCH) is a rare cancer
that begins in LCH cells
(a type of dendritic cell
which fights infection). Sometimes there are mutations
(changes) in LCH cells as they form. These include mutations of the BRAF
gene. These changes may make the LCH cells grow and multiply quickly. This causes LCH cells to build up in certain parts of the body, where they can damage tissue
or form lesions.
LCH is not a disease of the Langerhans cells that normally occur in the skin.
LCH may occur at any age, but is most common in young children. Treatment of LCH in children is different from treatment of LCH in adults. The treatments for LCH in children and adults are described in separate sections of this summary.
Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood Langerhans cell histiocytosis. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
Family history or having a parent who was exposed to certain chemicals may increase the risk of LCH.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for LCH include the following:
- Having a parent who was exposed to certain chemicals
such as benzene.
- Having a parent who was exposed to metal, granite, or wood dust in the workplace.
- A family history
of cancer, including LCH.
- Having infections as a newborn.
- Having a personal history
or family history of thyroid
- Smoking, especially in young adults.
- Being Hispanic.
The signs and symptoms of LCH depend on where it is in the body.
These and other signs
may be caused by LCH or by other conditions. Check with your doctor if you or your child have any of the following:
Skin and nails
LCH in infants may affect the skin only. In some cases, skin-only LCH may get worse over weeks or months and become a form called high-risk multisystem LCH.
In infants, signs or symptoms of LCH that affects the skin may include:
- Flaking of the scalp that may look like “cradle cap”.
- Raised, brown or purple skin rash anywhere on the body.
In children and adults, signs or symptoms of LCH that affects the skin and nails may include:
- Flaking of the scalp that may look like dandruff.
- Raised, red or brown, crusted rash in the groin
area, abdomen, back, or chest, that may be itchy.
- Bumps or ulcers
on the scalp.
- Ulcers behind the ears, under the breasts, or in the groin area.
- Fingernails that fall off or have discolored grooves that run the length of the nail.
Signs or symptoms of LCH that affects the mouth may include:
- Swollen gums.
- Sores on the roof of the mouth, inside the cheeks, or on the tongue or lips.
- Teeth that become uneven.
- Tooth loss.
Signs or symptoms of LCH that affects the bone may include:
- Swelling or a lump over a bone, such as the skull, ribs, spine, thigh bone, upper arm bone, elbow, eye socket, or bones around the ear.
- Pain where there is swelling or a lump over a bone.
Children with LCH lesions in bones around the ears or eyes have a high risk for diabetes insipidus
and other central nervous system
Lymph nodes and thymus
Signs or symptoms of LCH that affects the lymph nodes
- Swollen lymph nodes.
- Trouble breathing.
- Superior vena cava syndrome. This can cause coughing, trouble breathing, and swelling of the face, neck, and upper arms.
Signs or symptoms of LCH that affects the pituitary gland
- Diabetes insipidus. This can cause a strong thirst and frequent urination.
- Slow growth.
- Early or late puberty.
- Being very overweight.
Signs or symptoms of LCH that affects the thyroid
- Swollen thyroid gland.
- Hypothyroidism. This can cause tiredness, lack of energy, being sensitive to cold, constipation, dry skin, thinning hair, memory problems, trouble concentrating, and depression. In infants, this can also cause a loss of appetite
and choking on food. In children and adolescents, this can also cause behavior problems, weight gain, slow growth, and late puberty.
- Trouble breathing.
Central nervous system (CNS)
Signs or symptoms of LCH that affects the CNS (brain and spinal cord) may include:
- Loss of balance, uncoordinated body movements, and trouble walking.
- Trouble speaking.
- Trouble seeing.
- Changes in behavior or personality.
- Memory problems.
These signs and symptoms may be caused by lesions in the CNS or by CNS neurodegenerative syndrome.
Liver and spleen
Signs or symptoms of LCH that affects the liver
- Swelling in the abdomen caused by a buildup of extra fluid.
- Trouble breathing.
- Yellowing of the skin and whites of the eyes.
- Easy bruising or bleeding.
- Feeling very tired.
Signs or symptoms of LCH that affects the lung
- Collapsed lung. This condition can cause chest pain or tightness, trouble breathing, feeling tired, and a bluish color to the skin.
- Trouble breathing, especially in adults who smoke.
- Dry cough.
- Chest pain.
Signs or symptoms of LCH that affects the bone marrow
- Easy bruising or bleeding.
- Frequent infections.
Tests that examine the organs and body systems where LCH may occur are used to detect (find) and diagnose LCH.
The following tests and procedures may be used to detect (find) and diagnose
LCH or conditions caused by LCH:
Certain factors affect prognosis (chance of recovery) and treatment options.
LCH in organs such as the skin, bones, lymph nodes, or pituitary gland
usually gets better with treatment and is called "low- risk". LCH in the spleen, liver, or bone marrow is harder to treat and is called "high-risk".
(chance of recovery) and treatment options depend on the following:
- Whether there are mutations of the BRAF gene.
- How old the patient is when diagnosed with LCH.
- How many organs or body systems the cancer affects.
- Whether the cancer is found in the liver, spleen, bone marrow, or certain bones in the skull.
- How quickly the cancer responds
to initial treatment.
- Whether the cancer has just been diagnosed or has come back (recurred).
In infants up to one year of age, LCH may go away without treatment.
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