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. It may occur at any age, but is most common in young children.

Signs & Symptoms

The signs and symptoms of LCH depend on where it is in the body. These and other signs and symptoms may be caused by LCH or by other conditions. Check with your doctor if you have any of the following:

Skin and nails: In 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

Mouth: 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

Bone: 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 disease

Lymph nodes and thymus: Signs or symptoms of LCH that affects the lymph nodes or thymus may include:

  • Swollen lymph nodes
  • Trouble breathing
  • Superior vena cava syndrome: This can cause coughing, trouble breathing, and swelling of the face, neck, and upper arms.

Endocrine system: Signs or symptoms of LCH that affects the pituitary gland may include:

  • Diabetes insipidus: This can cause a strong thirst and frequent urination
  • Slow growth
  • Early or late puberty
  • Being very overweight

Thyroid: Signs or symptoms of LCH that affects the thyroid may include:

  • Swollen thyroid gland
  • 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 may include:

  • Loss of balance, uncoordinated body movements, and trouble walking
  • Trouble speaking
  • Trouble seeing
  • Headaches
  • 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 or spleen may include:

  • Swelling in the abdomen caused by a buildup of extra fluid
  • Trouble breathing
  • Yellowing of the skin and whites of the eyes
  • Itching
  • Easy bruising or bleeding
  • Feeling very tired

Lung: Signs or symptoms of LCH that affects the lung may include:

  • 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
  • Bone marrow

Bone marrow: Signs or symptoms of LCH that affects the bone marrow may include:

  • Easy bruising or bleeding
  • Fever
  • Frequent infections

Treatment

Your OHC doctor will help you determine the best care plan for you. There are nine types of standard treatment:

  • Chemotherapy uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing.
  • Surgery may be used to remove LCH lesions and a small amount of nearby healthy tissue. Curettage is a type of surgery that uses a curette (a sharp, spoon-shaped tool) to scrape LCH cells from bone. When there is severe liver or lung damage, the entire organ may be removed and replaced with a healthy liver or lung from a donor.
  • Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. In LCH, a special lamp may be used to send ultraviolet B (UVB) radiation toward LCH skin lesions.
  • Photodynamic therapy uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For LCH, laser light is aimed at the skin and the drug becomes active and kills the cancer cells. Photodynamic therapy causes little damage to healthy tissue. Patients who have photodynamic therapy should not spend too much time in the sun. In one type of photodynamic therapy, called psoralen and ultraviolet A (PUVA) therapy, the patient receives a drug called psoralen and then ultraviolet A radiation is directed to the skin.
  • Biologic therapy uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon is a type of biologic therapy used to treat LCH of the skin. Immunomodulators are also a type of biologic therapy. Thalidomide is an immunomodulator used to treat LCH.
  • Targeted therapy uses drugs or other substances to find and attack LCH cells without harming normal cells. Imatinib mesylate is a type of targeted therapy called a tyrosine kinase inhibitor. It stops blood stem cells from turning into dendritic cells that may become cancer cells. Other types of kinase inhibitors that affecT-cells with mutations (changes) in the BRAF gene, such as dabrafenib and vemurafenib, are being studied in clinical trials for LCH. A family of genes, called ras genes, may cause cancer when they are mutated. Ras genes make proteins that are involved in cell signaling pathways, cell growth, and cell death. Ras pathway inhibitors are a type of targeted therapy being studied in clinical trials. They block the actions of a mutated ras gene or its protein and may stop the growth of cancer.
  • Other drugs may be used.
  • Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the LCH treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.
  • Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change.