Eosinophilic granuloma
Overview
Histiocytosis is a general name for a group of syndromes that involve an abnormal increase in the number of immune cells called histiocytes. There are three major classes of histiocytoses: * Langerhans cell histiocytosis, which is also called histiocytosis X * Malignant histiocytosis syndrome (now known as T-cell lymphoma) * Non-Langerhans cell histiocytosis (also known as hemophagocytic syndrome) This article focuses only on Langerhans cell histiocytosis (histiocytosis X).
Symptoms
* Abdominal pain * Bone pain (possibly) * Delayed puberty * Dizziness Ear drainage that continues long-term Eyes that appear to stick out (protrude) more and more Irritability Failure to thrive Fever Frequent urination Headache Jaundice Limping
Causes
Histiocytosis X has typically been thought of as a cancer-like condition. More recently researchers have begun to suspect that it is actually an autoimmune phenomenon, in which immune cells mistakenly attack the body, rather than fight infections. Extra immune cells may form tumors, which can affect various parts of the body including the bones, skull, and other areas. Some forms of the disorder are genetic. Histiocytosis X is thought to affect roughly 1 in 200,000 people each year. It is most often seen in children age 1 to 15. The rate peaks among children age 5 to10.
Prevention
Avoid smoking. Quitting smoking can improve the outcome in people with histiocytosis that affects the lungs. There is no known prevention for the childhood forms of the disease.
Prognosis
Histiocytosis X affects many organs and can lead to death. About half of those with pulmonary histiocytosis see improvement, while others eventually have permanent loss of lung function. In very young patients, the outlook depends on the specific histiocytosis and severity of the disease. Some children can live a normal life with minimal disease involvement, while others may have a poor outcome. Young children, especially infants, are more likely to have body-wide symptoms that lead to death.
Treatment
This disorder is treated with corticosteroids, which suppress immune function (including the dangerous cells). Smoking may worsen the response to treatment and should be stopped. Children may be given other medications depending on their estimated outlook. Such medications may include: * Cyclophosphamide * Etoposide * Methotrexate * Vinblastine Radiation therapy or surgery may also be used to treat bone lesions.