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Langerhans Cell Histiocytosis (LCH)

Langerhans cell histiocytosis (LCH) is a very rare disease caused by the excessive growth of descendants of blood-forming cells, the LCH cells. LCH cells resemble Langerhans cells, immune cells that reside in the skin and protect the body against infections. In the event of Langerhans cell histiocytosis, the abnormal cells are usually located in groups in the bones and in the skin. The disease can also occur in other organs such as liver, spleen and intestine.

Symptoms

Because the disease can occur in bones, skin and various organs, it has many possible symptoms, including lumps and bone pain. Many patients also suffer from fever or skin lesions. Sometimes the disease is detected by chance on an X-ray taken for some other reason.

How is LCH diagnosed?

Peculiar round spots on bone X-rays might indicate Langerhans cell histiocytosis. A biopsy is taken in order to make a definitive diagnosis. The extent of the disease is recorded on X-rays of all the bones of the body. If vertebrae are involved, an MRI scan of the spine is also performed. If necessary, bone marrow is extracted and examined to determine whether LCH cells are also present in the bone marrow. Finally, there is the possibility of genetic testing to detect gene abnormalities in LCH cells.

Treatment

The intensity of the therapy depends on the extent of the disease. Treatment can range from a wait-and-see approach to mild or more intensive chemotherapy. Hopefully, drugs that target the LCH cells will be developed in the not too far future.

Questions?

Children and teenagers with LCH are treated in the hematology-oncology department.