Sometimes, I feel fine but then I get really sick and have to go to the hospital and have very strong treatments. My family joined a really good organization, the Histiocytosis Association of America, to help people and raise money for research. Although I’m doing well now, my mother says our family is “forever changed” by my rare disease.
Histiocytic disorders are a related group of diseases caused by over-production of white blood cells known as histiocytes. They can lead to organ damage and tumor formation. The most common disorder in this group is Langerhans cell histiocytosis (LCH).
Langerhans cells are a type of white blood cell that normally help the body fight infection. In LCH, too many of these cells are produced and build up in parts of the body where they can form tumors or damage organs.
Any age group can be affected, from infancy through adulthood. LCH is believed to occur at a rate of 1 in 200,000 children. Among very young infants, the disorder is thought to occur at a rate of 1 - 2 per million.
This condition is thought to be under-diagnosed since some people may have no symptoms while others have symptoms that are mistaken for injury or other conditions.
Symptoms depend on the location and severity of involvement. LCH in the skin, bones, lymph nodes or pituitary gland usually gets better with treatment and is considered low-risk. Involvement in the spleen, liver, bone marrow, lung and skeleton may be more difficult to treat.
While some cases of histiocytosis may be limited and may not require treatment, others may require chemotherapy.