Unifocal Langerhans-cell histiocytosis digital illustration

Unifocal Langerhans-cell histiocytosis Save

ICD-10 code: C96.6

Chapter: Neoplasms

Understanding Unifocal Langerhans-cell Histiocytosis: Symptoms, Causes, and Treatment

Unifocal Langerhans-cell histiocytosis (LCH) is a rare disease that affects the body's immune system. It is a type of histiocytosis where abnormal cells called Langerhans cells build up in one specific area of the body. This condition can occur in people of all ages but is most common in children under the age of 10.

Symptoms of Unifocal Langerhans-cell Histiocytosis

The symptoms of unifocal LCH depend on the location of the abnormal cells. The most common location for unifocal LCH is the bone. Symptoms of bone LCH can include pain, swelling, and limited movement of the affected bone. In some cases, bone LCH can lead to fractures. Other common locations for unifocal LCH include the skin, lungs, and lymph nodes. Symptoms of skin LCH can include a rash or lesions on the skin, while symptoms of lung LCH can include coughing, shortness of breath, and chest pain.

Causes of Unifocal Langerhans-cell Histiocytosis

The exact cause of unifocal LCH is not known. However, research suggests that it may be caused by a mutation in the BRAF gene, which is responsible for cell growth and division. This mutation causes an overgrowth of Langerhans cells, which can lead to unifocal LCH.

Treatment for Unifocal Langerhans-cell Histiocytosis

The treatment for unifocal LCH depends on the location of the abnormal cells and the severity of the symptoms. In many cases, observation and monitoring may be all that is needed, as some cases of unifocal LCH will resolve on their own. Other treatment options may include chemotherapy, radiation therapy, or surgery to remove the affected tissue.

  1. Chemotherapy: This treatment involves the use of drugs to kill the abnormal cells. Chemotherapy may be given orally or intravenously and can be administered on an outpatient basis.
  2. Radiation therapy: This treatment uses high-energy radiation to kill the abnormal cells. Radiation therapy may be used alone or in combination with other treatments.
  3. Surgery: In some cases, surgery may be necessary to remove the affected tissue. This is most commonly done in cases of bone LCH, where the affected bone may need to be removed and replaced with a bone graft.

Overall, unifocal Langerhans-cell histiocytosis is a rare condition that can be difficult to diagnose and treat. If you are

Diagnosis Codes for Unifocal Langerhans-cell histiocytosis | C96.6