The Langerhans cell histiocytosis (LCH) is a rare disease caused when an immune cell called Langerhans cells grow abnormally and accumulate in different parts of the body. In addition to causing damage to the skin, bones, lungs, liver, lymph nodes, brain, and spleen, LCH can also cause damage to other tissues and organs where the cells are found. Children are more likely to suffer from LCH than adults.
The exact cause of LCH is unknown, but it may be caused by a genetic mutation that occurs after birth in some Langerhans cells. The mutation causes the cells to multiply and invade the tissues where they are not supposed to be. LCH is not inherited, and it is not contagious. The following factors may increase the risk:
Smoking
Exposure to environmental toxins, such as benzene or wood dust
Infections in the newborn period
Family history of thyroid disease
LCH symptoms depend on the number, location, and size of Langerhans cell lesions in the body. Some people may not experience any symptoms, while others may have severe complications.
Skin rashes, especially in the scalp, groin, or armpits
Bone pain, swelling, or fractures
Ear infections or hearing loss
Eye problems, such as swelling, redness, or vision loss
Mouth ulcers or tooth decay
Swollen lymph nodes
Breathing difficulties or coughing
Abdominal pain or jaundice
Headaches, seizures, or behavioral changes
The diagnosis of LCH involves a physical examination and a biopsy of the affected tissues. During a biopsy, a small sample of tissue is taken and examined under a microscope for abnormal Langerhans cells. For confirmation of diagnosis and to determine the extent of the disease, the following tests may be performed:
Blood tests to check the levels of blood cells, liver enzymes, and thyroid hormones
Urine tests to check the levels of sugar and protein
X-rays, CT scans, MRI scans, or PET scans to get detailed images of the bones and organs
Bone marrow aspiration or biopsy to check the production of blood cells
The treatment of LCH depends on the severity, type, and location of the disease, as well as the age and general health of the person. The treatment options may include:
Observation or watchful waiting for mild or limited forms of LCH that may resolve on their own
Medications, such as steroids, chemotherapy, or targeted therapy, to reduce the inflammation and kill the abnormal cells
Surgery to remove the lesions or to repair the damaged bones or organs
Radiation therapy to shrink the lesions or to relieve the pain
Bone marrow transplant to replace the damaged bone marrow with healthy stem cells from a donor
Supportive care, such as pain relief, antibiotics, or nutritional supplements, to manage the symptoms and prevent infections or complications
The risk of complications associated with LCH cannot be prevented, since it is a genetic disorder that occurs randomly in some people. However, some steps can be taken to reduce the risk of complications and improve the quality of life of people with LCH.
Avoiding smoking or exposure to tobacco smoke or other harmful substances
Following the doctor’s recommendations for medication, surgery, or other treatments
Having regular check-ups and tests to monitor the condition and the response to treatment
Seeking support from family, friends, or professional counselors to cope with the emotional and physical challenges of living with LCH
