What is Langerhans Cell Histiocytosis?
I'm going to try to explain this as simple as possible.
Langerhans cells are a type of dendric cells of the skin. They can also be found in different parts of the body.
Histiocytes are cells that belong to the immune system. They are produced in the bone marrow along with white blood cells and red blood cells. They travel through your system to protect your body from infections. One type of these histiocytes are Langerhans cells.
Histiocytosis is the overproduction of histiocytes. Histocytosis is also an umbrella name for a group of rare diseases that share the same characteristics. Histiocytosis can attack any part of the body: skin, bones, muscles, organs (liver, kidneys, spleen, stomach, lungs, etc.) Histiocytosis also belongs to a family of other blood related diseases (diseases that derive from the bone marrow) such as leukemia, lymphoma, and erdheim-chester disease).
So combine the two, Langerhans cell histiocytosis (LCH) = the overproduction of Langerhans cells.
All types of histocytosis are very rare. LCH in particular, is most commonly found in children. The prevalence in adults is even more rare.
Cancer or Autoimmune disease?
Medical researchers have to date not agreed on how to classify LCH. Some argue that it is a cancer because it behaves and acts like a cancer. That is, the abnormal proliferation of cells which is by definition a tumor. Others claim however that it is an autoimmune disease because Langerhans cells are a part of your immune system. So, in this case the Langerhans cells are attacking your own body instead of foreign objects. The most recent research is leaning towards cancer however. But, some still stay firm claiming it's an autoimmune disease. Why people develop this disease is unknown. LCH in particular is a mystery and is commonly known as Histiocytosis X - cause unknown. Maybe that's why it's hard to define what it is.
Regardless of what it is, Oncologists (cancer specialists) and Hemotologists (blood specialists) treat this disease. Why? Read below...
There are several types of LCH.
Type I: unifocal. Benign. Affects only one part of your body, in one system. It is a slow progressing form. It usually involves bones, most often the skull. Can be solved easily with surgery and mild radiation therapy if necessary. In some cases doctors have found that the bone regresses and heals itself. Excellent prognosis.
Type II: multifocal unisystem. Malignant. Affects several parts of one system. For example, several area's of the bone. Other complications follow. Usually chemotherapy in combination with steroids is necessary to treat this type. Prognosis: around 60% chance of relapse.
Type III: multifocal multisystem. Aggressive malignanancy. Affects several systems of the body. If it affects your risk organs such as the liver or spleen, then the prognosis declines severley. This type progresses fast and should be treated with aggressive chemotherapy. The mortality rate is around 10%.
I was first diagnosed with type I. Then with the lesion in my hip, they diagnosed me with type II.
So why am I going through chemotherapy?
Because the lesion found in my hip developed within two months. This is fast progressing which could indicate I'm in a danger zone. If it managed to progress this fast within 2 months, it could mean that there's a high risk it will spread to other bone parts or in worse case, start developing in my organs (type III). So the solution is to start with chemo.
Hope this information helped :)
/H
Langerhans cells are a type of dendric cells of the skin. They can also be found in different parts of the body.
Histiocytes are cells that belong to the immune system. They are produced in the bone marrow along with white blood cells and red blood cells. They travel through your system to protect your body from infections. One type of these histiocytes are Langerhans cells.
Histiocytosis is the overproduction of histiocytes. Histocytosis is also an umbrella name for a group of rare diseases that share the same characteristics. Histiocytosis can attack any part of the body: skin, bones, muscles, organs (liver, kidneys, spleen, stomach, lungs, etc.) Histiocytosis also belongs to a family of other blood related diseases (diseases that derive from the bone marrow) such as leukemia, lymphoma, and erdheim-chester disease).
So combine the two, Langerhans cell histiocytosis (LCH) = the overproduction of Langerhans cells.
All types of histocytosis are very rare. LCH in particular, is most commonly found in children. The prevalence in adults is even more rare.
Cancer or Autoimmune disease?
Medical researchers have to date not agreed on how to classify LCH. Some argue that it is a cancer because it behaves and acts like a cancer. That is, the abnormal proliferation of cells which is by definition a tumor. Others claim however that it is an autoimmune disease because Langerhans cells are a part of your immune system. So, in this case the Langerhans cells are attacking your own body instead of foreign objects. The most recent research is leaning towards cancer however. But, some still stay firm claiming it's an autoimmune disease. Why people develop this disease is unknown. LCH in particular is a mystery and is commonly known as Histiocytosis X - cause unknown. Maybe that's why it's hard to define what it is.
Regardless of what it is, Oncologists (cancer specialists) and Hemotologists (blood specialists) treat this disease. Why? Read below...
There are several types of LCH.
Type I: unifocal. Benign. Affects only one part of your body, in one system. It is a slow progressing form. It usually involves bones, most often the skull. Can be solved easily with surgery and mild radiation therapy if necessary. In some cases doctors have found that the bone regresses and heals itself. Excellent prognosis.
Type II: multifocal unisystem. Malignant. Affects several parts of one system. For example, several area's of the bone. Other complications follow. Usually chemotherapy in combination with steroids is necessary to treat this type. Prognosis: around 60% chance of relapse.
Type III: multifocal multisystem. Aggressive malignanancy. Affects several systems of the body. If it affects your risk organs such as the liver or spleen, then the prognosis declines severley. This type progresses fast and should be treated with aggressive chemotherapy. The mortality rate is around 10%.
I was first diagnosed with type I. Then with the lesion in my hip, they diagnosed me with type II.
So why am I going through chemotherapy?
Because the lesion found in my hip developed within two months. This is fast progressing which could indicate I'm in a danger zone. If it managed to progress this fast within 2 months, it could mean that there's a high risk it will spread to other bone parts or in worse case, start developing in my organs (type III). So the solution is to start with chemo.
Hope this information helped :)
/H
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