What is the meaning of histiocytosis?
Listen to pronunciation. (HIS-tee-oh-sy-TOH-sis) A group of rare disorders in which too many histiocytes (a type of white blood cell) build up in certain tissues and organs, including the skin, bones, spleen, liver, lungs, and lymph nodes.
What are the symptoms of histiocytosis?
The first sign of histiocytosis is often a rash on the scalp, similar to cradle cap. There may be a pain in a bone, discharge from the ear, loss of appetite and fever. Sometimes the stomach is swollen and painful.
Is Langerhans cell histiocytosis a form of leukemia?
Acute lymphoblastic leukemia is more often associated with LC histiocytosis in children, whereas lymphoma and acute myeloid leukemia are more common in adults, which may reflect the frequency of these diseases in the respective populations.
Is there a cure for histiocytosis?
Radiation therapy can be extremely effective for certain types of histiocytosis, especially Langerhans cell histiocytosis. Radiation is often recommended to treat people whose histiocytosis is affecting only one area of the body.
How is histiocytosis diagnosed?
Radiology Scans CT scans can identify areas of histiocytosis. They can be used to look at any part of the body, but CT is most helpful for detecting histiocytosis in the lungs, heart, kidneys, lymph nodes, and other organs. MRI scans can get a very detailed picture of certain organs.
What is histiocytosis in adults?
Langerhans cell histiocytosis (LCH) in adults is a rare disorder that occurs when the body produces too many Langerhans cells (histiocytes), which are a type of white blood cell that helps fight infection.
Is LCH life threatening?
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.
Does histiocytosis go away?
In many people with Langerhans cell histiocytosis, the disorder eventually goes away with appropriate treatment. It may even disappear on its own, especially if the disease occurs only in the skin.
What is the survival rate of LCH?
What are the survival rates for Langerhans cell histiocytosis? About 99% of children with low-risk LCH survive. About 80% of children with high-risk LCH survive.
Is histiocytosis fatal?
Langerhans cell histiocytosis can cause damage to tissues and organs all over the body if it’s not treated. One example is pulmonary histiocytosis. This condition damages the lungs. Damage to the body can be so severe that the condition becomes fatal.
What is PLCH?
Pulmonary Langerhans cell histiocytosis (PLCH), previously called eosinophilic granuloma of the lung, pulmonary Langerhans cell granulomatosis, and pulmonary histiocytosis X, is an uncommon cystic interstitial lung disease that primarily affects young adults [1-5]. It is caused by a disorder of myeloid dendritic cells.
Is histiocytosis 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.
What does histiocytosis stand for in medical category?
Histiocytosis is a general name for a group of disorders or “syndromes” that involve an abnormal increase in the number of specialized white blood cells that are called histiocytes. Recently, new knowledge about this family of diseases has led experts to develop a new classification. Five categories have been proposed:
What do you need to know about Langerhans cell histiocytosis?
People with widespread Langerhans cell histiocytosis or Erdheim-Chester disease require medicines to reduce symptoms and control the spread of the disease. Recent studies show that nearly all adults with widespread histiocytosis have gene mutations in the tumors, which appear to cause the disorder.
Which is the most common histiocytic disorder in adults?
Among primary histiocytic disorders, pulmonary Langerhans cell histiocytosis (PLCH) is the most common; others include Erdheim-Chester disease and Rosai-Dorfman disease. Adult PLCH occurs almost exclusively in adults aged 20–40 years who smoke.
Are there any treatments for juvenile histiocytosis?
Some forms of RDD do not require treatment and can improve on their own. For those that do not, treatments include surgery, steroid medications, and in some cases, chemotherapy or targeted drugs. Juvenile xanthogranuloma (JXG) is a histiocytosis that occurs in children and adolescents.