Langerhans Cell Histiocytosis

Langerhans cell histiocytosis, also called histiocytosis X, is a condition in which the level of a type of immune cell, called a Langerhans cell, is abnormally high. While Langerhans cell histiocytosis has been considered to be a type of cancer or a condition similar to cancer, researchers are now discovering that it is more likely tied to an autoimmune response and occurs when the body's immune system attacks itself.

Facts about Langerhans cell histiocytosis

Langerhans cell histiocytosis occurs in about one out of every 200,000 people. It most often strikes young children. Langerhans cell histiocytosis causes damage to tissues throughout the body. The excess Langerhans cells, which are a type of white blood cell, flow throughout the body and build up in areas such as the skin, bones, bone marrow, lymph nodes, spleen, liver, lungs, pituitary gland, and central nervous system, creating tumors.

Symptoms

The symptoms of Langerhans cell histiocytosis depend on where in the body the Langerhans cells build up. These are possible symptoms:

• Pain in the abdomen or in the bones

• Poor growth or failure to thrive

• Irritability

• Jaundice, or yellowing of the skin and eyes

• Urinating frequently and having constant thirst (caused by diabetes insipidus)

• Dark red or brown lesions on the skin, especially on the face, scalp, back, and groin

• Bulging eyes

• Persistent drainage from the ear

• Headaches

• Flaking skin on the scalp similar to cradle cap

• Problems with the thyroid

• Problems with balance, memory, or behavior

Diagnosis

A doctor may diagnose Langerhans cell histiocytosis through a combination of tests, including:

• Complete physical examination

• Discussion of symptoms or illnesses

• Neurological examination to evaluate the spinal cord, brain, nerves, and mental functioning

A number of lab tests may also be performed to diagnose the condition, including blood tests, urine tests, and a biopsy of the bone marrow. Imaging tests, including a bone scan, CT scan, MRI, ultrasound, and PET scan may also be performed.

Treatment

Treatment for Langerhans cell histiocytosis typically includes those designed to treat cancer:

• Chemotherapy

• Radiation therapy

• Surgery to remove abnormal tissues

• Corticosteroid drugs

• Photodynamic therapy

Some patients with Langerhans cell histiocytosis may opt for "watchful waiting," in which the symptoms and progress of the condition are closely monitored but active treatment is postponed until there is a change in the condition.

Calling the doctor

Parents should monitor children carefully and look for any warning signs of Langerhans cell histiocytosis, since the condition is most common in kids. Bring up any concerns, growth problems, or unusual symptoms to your child's pediatrician.

Prevention

Because the cause of Langerhans cell histiocytosis is unknown, there is no known way to prevent the condition. Having a family history of thyroid disease may increase the risk.

Some factors can increase a child's risk of developing the condition: Parents should limit their children's exposure to certain chemicals, including benzene.

Complications

If untreated, Langerhans cell histiocytosis can cause damage to tissues and organs throughout the body. One example is pulmonary histiocytosis, in which the lungs are damaged. Damage to the body can be so severe that the condition becomes fatal. Other complications may include:

• Pituitary gland problems

• Growth problems (including delayed growth)

• Anemia

• Lung failure

Key points to remember

Langerhans cell histiocytosis is a rare condition. Early recognition of symptoms and prompt diagnosis and treatment can help reduce the risk for complications and slow the progression of the disease. Clinical trials are being performed to find new, better ways to treat this condition.