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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 Langerhans Cell Histiocytosis the abnormal cells are found in groups mostly in the bones and in the skin. The disease can also occur in other organs such as liver, spleen and intestine.

Symptoms of this disease

Because the disease can occur in bones, skin and various organs, the possible symptoms are extensive. There is often a lump or pain in a bone. Patients often also suffer from fever or skin abnormalities. Sometimes the disease is discovered by chance because it is detected on an X-ray that was made for a different reason.

How do we make the diagnosis?

Typical round spots on X-rays of a bone may indicate a Langerhans Cell Histiocytosis. To definitively determine the disease, a biopsy is taken. 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 made. A bone marrow puncture will, if necessary, be examined to see whether LCH cells are also present in the bone marrow. Finally, genetic research is possible to demonstrate gene abnormalities in LCH cells.

Treatment of LCH

The intensity of the therapy depends on the extent of the disease. This can vary from a wait-and-see policy to mild or more intensive chemotherapy. Hopefully, drugs will be developed in the future that will single out the LCH cells in a targeted manner.