Tuesday, September 9, 2008
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Max has Langerhans cell histiocytosis (LCH)
Disease Information
Histiocytosis is a rare blood disease that is caused by an excess of white blood cells called histiocytes. The histiocytes cluster together and can attack the skin, bones, lung, liver, spleen, gums, ears, eyes, and/or the central nervous system. The disease can range from limited involvement that spontaneously regresses to progressive multiorgan involvement that can be chronic and debilitating. In some cases, the disease can be life-threatening.
In some ways, histiocytosis is similar to cancer and has historically been treated by oncologists with chemotherapy and radiation. Unlike cancer, histiocytosis sometimes goes into remission without treatment.
The vast majority of people diagnosed with histiocytosis are children under the age of 10, but it is also found in adults of all ages.
It is approximated that histiocytosis affects 1 in 200,000 children born each year in the United States. This illness is so rare, there is little research into its cause and treatment, and it is often referred to as an "orphan disease," meaning it strikes too few people to generate government - supported research.
LCH in Children
Langerhans cell histiocytosis (LCH) is a rare disorder that primarily affects children. The disease was first described in medical literature around the turn of the 20th century.
A histiocyte is a form of white blood cell. Its job is to help destroy certain foreign materials and fight infection. For unknown reasons, patients with this disease have too many histiocytes (Langerhans cells). These cells accumulate in different organs and can result in a variety of symptoms.
Langerhans cell histiocytosis has also been known as Histiocytosis-X, Eosinophilic Granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian Syndrome.
There are also a number of other terms which have been used to describe syndromes which are considered to be Langerhans cell histiocytosis (LCH). These include: Reticuloendotheliosis, Hasimoto-Pritzker syndrome, Self-healing histiocytosis, Pure cutaneous histiocytosis, Langerhans cell granulomatosis, Type II histiocytosis, and Non-lipid reticuloendotheliosis.
The cause of LCH is unknown. It may be triggered by an unusual reaction of the immune system from something commonly found in the environment. It is not a known infection or cancer. It is not known to be hereditary or communicable.
Over the years, cancer treatments have been used in patients with histiocytosis. Consequently, hematologists and oncologists, who treat cancer, also treat children with Langerhans cell histiocytosis. However, the disease is not cancer. Radiation therapy, if used, is given in much lower doses than that which cancer patients receive.
The vast majority of patients will survive the disease. Some may develop life-long chronic problems, while others remain symptom free. In some cases the disease is fatal. Usually these are very young infants who have a rapid downhill course and do not respond to any known treatment. Whether or not the disease responds to treatment will often depend on the extent of organ involvement; however, it is often difficult to make definite predictions. Physicians will be able to discuss the patient's likelihood of responding to treatment and doing well.
It is estimated that 8.9 of every 1,000,000 children under the age of 15 have histiocytosisSeventy-six (76) percent of the cases occur before ten (10) years of age, but the disease is also seen in adults of all ages.
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