Disease Information
Leukemias / Lymphomas: Langerhans Cell Histiocytosis
Alternate Names: LCH, Hand-Schuller-Christian syndrome, Letterer-Siwe disease, eosinophilic granuloma
Definition
Langerhans Cell Histiocytosis (LCH) is a rare, proliferative disorder of dendritic cells. Dendritic cells are a type of immune cell that can be found in various tissues in the body. In LCH, the dendritic cells (also called Langerhans cells or histiocytes) multiply and cause damage to the tissue where they are located. The clinical picture of LCH can vary widely. Often, it involves only a single organ such as skin, bone, or lymph nodes. Other patients can present with multi-system disease involving any organ of the body, most commonly bone marrow, lungs, liver and brain. While LCH is not a cancer, it typically requires treatment with chemotherapy similar to that used to treat many childhood cancers.
Incidence
- Approximately two to three out of every one million children will develop Langerhans cell histiocytosis every year
- The most common age to develop LCH is 2 to 3 years old; however, it can be diagnosed in children at birth and into the teenage years.
Influencing Factors
There is no known associated risk-factor for pediatric LCH, although research has suggested infectious and environmental triggers for the disease. Cigarette smoking is clearly linked with increased risk for pulmonary LCH in adults.
Survival Rates
- Patients with low-risk LCH (skin or bone only, or multiple organs not including the liver, lung, bone marrow, or spleen) have an excellent prognosis with approximately 99% survival.
- High-risk LCH, defined as multi-system disease involving liver, lung, bone marrow, or spleen has a survival rate of about 80%.
Treatment Strategies
Patients with only skin involvement will occasionally have improvement without therapy. Single bone lesions can often be cured with surgical removal followed by observation. More extensive disease requires treatment with chemotherapy for a duration of about 1 year.
Current Research
- Researchers are looking ways to improve the prognosis of patients with risk-organ involvement and of patients who do not respond to initial therapy.
- New chemotherapeutic agents are being studied to determine their effectiveness in treating LCH.
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