What is multisystem Langerhans cell histiocytosis?

What is multisystem Langerhans cell histiocytosis?

Langerhans cell histiocytosis is a rare disorder that can damage tissue or cause lesions to form in one or more places in the body. It is not known whether LCH is a form of cancer or a cancer-like disease.

Is Langerhans cell histiocytosis life threatening?

The prognosis for children with LCH is generally excellent. The disease is rarely life-threatening. However, some LCH survivors experience long-term effects, such as orthopedic disabilities, hearing impairment, diabetes insipidus, and skin scarring.

What causes Langerhans cell histiocytosis?

The cause of this disease is unknown, although most data suggest that it is characterized by a growth of immature Langerhans cells that appear to have genetic changes of the BRAF gene in about half the cases. LCH is not caused by a known infection, is not contagious, nor is it believed to be inherited.

What are the symptoms of Langerhans cell histiocytosis?

Other signs and symptoms that may occur in Langerhans cell histiocytosis, depending on which organs and tissues have Langerhans cell deposits, include swollen lymph nodes, abdominal pain, yellowing of the skin and whites of the eyes (jaundice), delayed puberty, protruding eyes, dizziness, irritability, and seizures.

Is LCH a leukemia?

Langerhans cell histiocytosis (LCH) is a non-malignant disorder, whether localized or disseminated, and usually has a favourable prognosis. A possible relationship between LCH and neoplastic diseases has not been assessed up to now even if a few cases have been recorded.

What are the symptoms of Langerhans Cell Histiocytosis?

Is LCH an autoimmune disease?

Langerhans cell histiocytosis historically was thought of as a cancer-like condition, but more recently researchers have begun to consider it an autoimmune phenomenon in which immune cells begin to overproduce and attack the body instead of fighting infection.

Is histiocytosis an autoimmune disease?

Are you born with LCH?

LCH symptoms can range from mild to more serious. Some people are born with it, and the disease eventually goes away on its own. But others have a severe and long-lasting type that affects multiple parts of the body.

Is LCH fatal in adults?

Is LCH fatal? It can be. A small percentage of patients, most often those with multisystem risk-organ involvement that is unresponsive to treatment, may not survive.