What is Langerhans Cell Histiocytosis?

You may know that white blood cells are your body’s infection fighters. But if your body makes too much of a type of immune cell called histiocytes, they can cause tumors, lesions, and other tissue damage.

One of these rare disorders -- which resembles some types of cancer -- is called Langerhans cell histiocytosis, or LCH. It most commonly appears in toddlers and very young children, but you can have it as an adult, too.

Symptoms

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.

Along with your bones, it can affect your:

Skin. Red, scaly bumps in skin folds are common. Infants with LCH can get red, scaly scalps, which is often mistaken for cradle cap, a common skin condition.

Liver. Usually, only severe cases of LCH affect the liver. Your skin might appear jaundiced, or yellow, and your blood can take longer to clot.

Lymph nodes. These glands, which are behind the ears, in the neck, and in other places, can swell up. You also might have trouble breathing or have coughs.

Diagnosis

Your doctor will know if you have LCH after a tissue biopsy. That’s when a specialist called a pathologist views the sample under a microscope for specific proteins and other markers of the disease.

In addition to the physical exam, your doctors may order other tests based on your symptoms:

• X-rays of your lungs and bones
• Bone marrow biopsy to look for signs of LCH
• Blood chemistry test to check your kidneys, liver, thyroid, and immune system
• MRIs and PET and CT scans to get detailed images of your body
• Urinalysis to check levels of red and white cells, protein, and sugar in your pee

Causes

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.

Researchers suspect that other things may also 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

Treatments

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.

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