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Langerhans cell histiocytosis (LCH) is a rare disorder that causes over-production of histiocytes, a type of immune cell (infection-fighting cell) that is present within body tissues. Histiocytosis can lead to tumor formation and organ damage.
Normally, histiocytes help guard the body against infection. Histiocytosis occurs when large numbers of these immune cells grow out of control.
There are three major classes of histiocytosis, and each class represents a very different medical condition. LCH is the most common form of the condition and usually affects young children. The other two classes are non-Langerhans cell histiocytosis (non-LCH) and malignant histiocytosis. These occur much less frequently. One form of non-LCH is hemophagocytic lymphohistiocytosis (HLH) lymphohistiocytosis (HLH).
The cause of most cases of histiocytosis is unknown. It is thought that it may be an environmental trigger that results in an unusual reaction from the immune system. Some forms of histiocytosis are hereditary (passed down from parent to child). LCH has recently been shown to display genetic changes found in certain cancers.
LCH causes a wide range of symptoms which can include:
LCH is sometimes associated with the following:
A biopsy of tissue affected by LCH allows doctors to diagnose this disorder. A biopsy is a small bit of tissue taken from the tumor. It is examined under a microscope to determine if the cells of the tumor have the size, shape and specific proteins found in histiocytic tumors.
Doctors at Riley at IU Health may also have other tests done to determine the diagnosis of LCH. These tests include:
In rare, specific situations, LCH will go away without treatment. However, most patients will need therapy for the histiocytosis to go away. The specific therapy used to treat histiocytosis will depend on factors such as:
Available treatments include:
Most children with LCH can be treated successfully and remain healthy after treatment.
Visit the online sources listed below to learn more about LCH.
Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.
The Histiocytosis Association is a global organization that helps patients and families learn to manage histiocytosis and supports research for a cure.
The National Cancer Institute provides information about LCH including treatments, causes and prevention, screenings and general coping resources.
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