Langerhans Cell Histiocytosis

________________________________________________

What is cancer?


Cancer is disease caused by an uncontrolled division of abnormal cells in a part of the body.

A specific cancer I will be informing you about today is Langerhans Cell Histiocytosis or (LCH)

Langerhans cell histiocytosis (LCH) is a rare cancer that begins in LCH cells, a type of dendritic cell (white blood cell ). LCH cells can grow in many different parts of the body, where they can damage tissue or form lesions.

Which part of the body does this type of cancer affect?

LCH is most commonly found in the skin, bones, lymph nodes, or pituitary glands.


What specific things/exposures can lead to this type of cancer?

Langerhans cell histiocytosis (LCH) is the most common of the histiocytic disorders and occurs when the body accumulates too many immature Langerhans cells, a subset of the larger family of cells known as histiocytes. Langerhans cells are a type of white blood cell that normally help the body fight infection. In LCH, too many Langerhans cells are produced and build up in certain parts of the body where they can form tumors or damage organs. The cause of this disease is unknown, although many possibilities have been explored, including viruses, exposure to toxins in the environment, family history and geography.


What are the symptoms, how do you know you have it? What are the effects?

  • Pain, swelling or lump in a bone that does not go away

  • Broken bone for no clear reason or from only a minor injury

  • Loose teeth when you would not expect this, or swollen gums

  • Ear infection, cysts in the ear or fluid that oozes from the ear

  • Skin rash, such as on the scalp or buttocks

  • Swollen lymph nodes in the neck (also called swollen glands)

  • Liver problems, which may cause jaundice (yellow color in the whites of the eyes; may be a yellow tint in the skin for some skin colors), fluid in the belly, diarrhea or vomiting

  • Bulging eyes or other eye problems

  • Cough and trouble breathing

  • Weight loss for no reason

  • Not gaining weight and not growing normally (failure to thrive)

  • Not wanting to eat or having problems feeding

  • Needing to urinate more often than normal and being very thirsty (signs of a health problem called diabetes insipidus)

  • Basic signs of illness, such as fever, fatigue and weakness

What are the treatments, what is the survival rate for people with this type of cancer?

No consensus exists for the optimal therapy for Langerhans cell histiocytosis (LCH), particularly in the case of multi system organ disease. However, the Histiocyte Society has conducted a number of prospective, randomized control trials to study the effect of various chemotherapeutic regimens in the treatment of LCH, which have resulted in recommendations by the Histiocyte Society.

About 99 percent of children with low-risk LCH survive. The survival rate for high-risk LCH is about 80 percent.

Langerhans Cell Histiocytosis