Langerhans cell histiocytosis - explain?
My friend has been told that his 4-year-old son has Langerhans cell histiocytosis (single system). What treatment, if any should he have?
A histiocyte is a special cell-type that is found in the bone marrow, blood, skin, lymph glands and the spleen. Sometimes histiocytes can migrate into other tissues of the body and can cause damage to those tissues. Single system disease means that the disease affects just one part of the body as opposed to multi system disease where several parts of the body may be affected. The name Langerhans simply refers to the doctor who first described this condition. The condition is very rare and its cause is unknown. The pattern of presentation can be very variable and diagnosis is usually based on tissue biopsy, bone x-rays and blood tests. Most cases of single system disease resolve spontaneously without treatment whereas those with multi-system disease may need chemotherapy. Langerhans cell histiocytosis can recur so it is necessary for affected children to be reviewed periodically by a paediatrician. In conclusion those with single system disease generally have an excellent prognosis.