About type ii histiocytosis
What is type ii histiocytosis?
Langerhans cell histiocytosis (LCH) is a spectrum of rare disorders characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the various tissues and organs of the body (lesions). The lesions may include certain distinctive Langerhans cells involved in certain immune responses, as well as other white blood cells (e.g.,lymphocytes, monocytes, eosinophils). Associated symptoms and findings may vary from case to case, depending upon the specific tissues and organs affected and the extent of involvement. Most often the bone lesions are painful. Skin rashes may itch or cause painful ulcers especially under the arms or groin area. The pathogenesis (medical cause) is not clearly understood and an ongoing debate continues regarding its cause as a reactive immunologic or neoplastic (cancer-like) process. No infectious agent (virus, bacteria, or fungus) has been associated with LCH. Patients often have a strong family history of immune diseases such as thyroid disease, arthritis, or lupus.
Most affected individuals have single or multiple bone lesions characterized by lytic lesions (holes in the bones). Although the skull is most commonly affected, there may also be involvement of other bones, such as those of the spine (vertebrae) and the long bones of the arms and legs. Affected individuals may have no apparent symptoms (asymptomatic), or may experience associated pain and swelling, and/or develop certain complications, such as fractures or secondary compression of the spinal cord. Other organs may also be affected, including the skin, lungs, liver, spleen, bone marrow, thymus, thyroid,intestines and brain. In some individuals, LCH may be associated with involvement of the pituitary gland leading to diabetes insipidus, growth failure, hypothyroidism, or insufficitne production of sex hormones.
Langerhans cell histiocytosis was selected by the Histiocyte Society to replace the older, less specific term histiocytosis X. Histiocytosis X encompassed three entities known as eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease that were characterized by the accumulation of histiocytes. The "X" denoted that the cause and development of the disorder was not understood. Langerhans cell histiocytosis was chosen because it seemed that the Langerhans cells might play a central role in the development of these disorders. However, new research (Allen 2010) has shown that the skin Langerhans cell is not the cell of origin, but a myeloid dendritic cell.
What are the symptoms for type ii histiocytosis?
LCH can show up on many places on your body, but most often on your skin and bones. It can affect any organ, including your lungs, liver, brain, spleen, or lymph nodes.
In about 8 out of 10 people, LCH leads to tumors called granulomas in the skull and in other bones. That can cause Pain and swelling, and sometimes it can fracture your arms or legs.
LCH symptoms can range from mild to more serious. Some people are born with it, and the disease eventually goes away on its own. But others have a severe and long-lasting type that affects multiple parts of the body.
What are the causes for type ii histiocytosis?
We don’t know all the reasons why some people get LCH. About half of the people with the disorder have a faulty gene that makes the Langerhans immune cells grow out of control. That genetic mutation happens after birth, which means you usually won’t get LCH from your parents.
What are the treatments for type ii histiocytosis?
As with some types of cancer, doctors sometimes treat LCH with chemotherapy. Many people with the disorder get care from cancer specialists like oncologists and haematologists. But unlike most cancers, limited forms of LCH sometimes spontaneously go away on their own.
Beside chemotherapy, options for treatment include:
- Low-dose radiation to targeted part of the body
- Surgery to remove LCH lesions
- Steroids like prednisone or anti-inflammatory drugs
- Ultraviolet light therapy for skin conditions
- Stem cell transplant
- Bone marrow, liver, or lung transplants in very serious cases
A great majority of people with LCH recover with treatment. If the disease is in your spleen, liver, or bone marrow, it’s called a high-risk LCH. About 80% of people with that type survive.
What are the risk factors for type ii histiocytosis?
Researchers suspect these things play a role:
- Smoking
- Parents who were exposed to environmental toxins, like benzene or wood dust
- Infections as a newborn
- Family history of thyroid disease