What is Langerhans Cell Histiocytosis (Histocytosis X)?

Langerhans cell (not to be confused with Islets of Langerhans in the pancreas) histocytosis, also known as Histocytosis X, is a condition that looks and acts like a type of cancer; at one time, it was thought that this disease was a form of cancer. Now it is believed to be an autoimmune disorder. Rather than fighting diseases, the cells of the immune system attack the body. The immune system mistakenly creates an overabundance of specialized white blood cells, which end up causing tumors in the body. The tumors can form on the bones and the soft tissues of the body. Langerhans cell histocytosis can occur in adults and children. The disease occurs in 1 out of 200,000 people annually. The disease can affect any area of the body.

If the disease attacks the lungs, it is called pulmonary histocytosis X. The disease causes stiffening of the lungs; the small airways of the lungs become inflamed and swollen, thereby making the airways smaller. The blood flow to the lungs can also be compromised due to the inflammation and swelling in the small blood vessels that supply circulation to the bronchioles (tiny airways in the lungs).

Symptoms of Langerhans cell histocytosis

The symptoms of Langerhans cell histocytosis can affect the entire body; when this happens it is called systemic Histocytosis X. The symptoms may vary from person to person. Some of the common symptoms of Langerhans cell histocytosis include:

Bone pain

Abdominal pain

Chest pain

Coughing

Shortness of breath

Dizziness

Delayed puberty

Protruding eyes

Draining of the ears

Fatigue

Fever

Failure to thrive in children

Headache

Frequent urination

Limping

Jaundice

Red or purple colored rashes

Greasy, flaky condition of the scalp

Shortness of stature

Seizure activity

Nausea and vomiting

Thirst

Weight loss

In patients where the Langerhans cell tumors grow in the weight bearing bones of the body, the bones may be at risk for fracturing without any warning. Children who have this disease may have only bone involvement and not lung involvement. Adults having Langerhans cell histocytosis may have more of the symptoms listed above.

How is Langerhans cell histocytosis diagnosed and treated?

The doctor will get a medical history from the patient, and then do a physical examination. An X-ray will show the tumors on the bones which appear to be black spaces, like punched out holes in the bones. The doctor may also collect a biopsy of the skin and the bone marrow. In both biopsies the doctor will look for the presence of Langerhans cells. Langerhans cells are normally found in the lymph nodes and some of the organs in the body. A complete blood count (CBC) will be done to give the doctor a picture of the immunity cells within the blood.

Other tests the doctor may need to do will evaluate the lungs and lung function. These tests include chest X-rays, pulmonary function tests and a bronchoscopy and biopsy. With a bronchoscopy, a tube is inserted down into the trachea; a biopsy will be taken from the internal parts of the respiratory system at the time of the bronchoscopy.

Adults are treated with corticosteroids, which suppress the immune system. By suppressing the immune system, the immune cells that cause the disease are suppressed also. It is important that people refrain from smoking when they are being treated for Langerhans cell histocytosis, because smoking can interfere with the body's ability to respond to the treatment.

Children may be treated with corticosteroids and also other drugs such as methotrexate, cyclophosphamide, etoposide, and vinblastine. Radiation therapy and surgery may also be indicated for some patients.

Conclusion

Adults and children being treated for Langerhans cell histocytosis may also be treated with antibiotics to combat infections. People who have seborrhea type conditions of the scalp may be helped with specialized shampoos designed to treat this condition. In some cases, the patient may need breathing support. Pain medications may be needed to relieve the aches and pains of having this disorder. Other treatments may include hormone replacement therapy and physical therapy.

Sources:

Medline Plus

Web MD