Heart and Blood Conditions

Langerhans Cell Histiocytosis

What is Langerhans cell histiocytosis?

In Langerhans cell histiocytosis, certain white blood cells (Langerhans cells) grow out of control. In a healthy person, Langerhans (LANG-er-hanz) cells help fight infection. But in histiocytosis (HIS-tee-oh-sy-TOE-sis), the cells grow so quickly that they build up. This can damage the body instead of protecting it.

Some children with the disease have extra cells and damage in only 1 area or organ in their bodies. In other children, extra cells build up in many places. Langerhans cell histiocytosis is most likely to damage bones, but it can affect many body systems.

A person of any age can get histiocytosis, but it is most common in children 1 to 3 years old. Most people with the disease get it before age 10.

  • Doctors do not know what causes this disease. It is not inherited and is not spread from person to person.

    Like cancer, cells grow too quickly. In some children with the condition, the young Langerhans cells have genetic changes. Although this condition is not cancer, doctors treat it in the same ways as they treat cancer.

  • This condition has had other names, and there are subtypes of the disease. You may hear or see these other names:

    • Diffuse reticuloendotheliosis
    • Eosinophilic granuloma
    • Hand-Schüller-Christian disease
    • Hashimoto-Pritzker syndrome
    • Histiocytosis X
    • Langerhans cell granulomatosis
    • Letterer-Siwe disease
    • Nonlipid reticuloendotheliosis
    • Type II histiocytosis

Langerhans Cell Histiocytosis at Seattle Children’s

This is a rare disease, and not many doctors have experience treating it. Our doctors do. At Seattle Children’s Cancer and Blood Disorders Center, we have cared for many children with histiocytosis and can offer the latest treatments for your child.

Knowing what to watch for helps us prevent and treat problems early. This increases the chances of successful treatment and helps your child feel better.

Please contact the center at 206-987-2106 for more information, a second opinion or to make an appointment.

  • Our doctors are nationally known for treating children who have blood disorders. The doctors who guide your child’s care specialize in pediatric hematology-oncology. This means they are specially trained to give the expert care your child needs, and they constantly expand their knowledge about blood disorders and cancer.

    Our experience helps us recommend the right treatment at the right time to have the best results for your child and your family.

    We keep a close watch on your child’s health and provide treatments as needed. The options include surgery, chemotherapy and other medicines, radiation therapy and, in rare cases, stem cell transplant.

    Seattle Children’s has the experts to treat health problems this condition may cause. It could affect your child’s growth, bones, skin, hormones, lungs or liver. Regular checkups and timely treatment can help your child live a healthy, active life.

  • Our specialty is treating children’s conditions while helping them grow up to be healthy and productive. In 2017, U.S. News & World Report ranked Seattle Children’s the top pediatric hospital in the Northwest and among the best in the nation.

    Children don’t react to illness, injury, pain and medicine in the same way as adults. They need – and deserve – care designed just for them.

    Our doctors have special training in how to diagnose and treat children with blood disorders. Our experts base their treatment plans on years of experience and the newest research on what works best – and most safely – for children.

    We care for your whole child, not just their disease. Your family has a full team behind you made up of doctors, nurses and other specialists as needed. Your child’s team may include experts in nutrition, pain control, physical therapy and emotional health. Read more about the supportive care we offer.

  • Having a child with Langerhans cell histiocytosis can be stressful. We offer same-day visits for children with urgent needs. New patients whose needs are not urgent usually can be seen within 1 or 2 weeks.

    During visits, we take time to explain your child’s condition. We help you fully understand your treatment options and make the choices that are right for your family.

    Our child life specialists and social workers help your child and your family through the challenges of this condition. We connect you to community resources and support groups.

    At Seattle Children’s, we work with many children and families from around the Northwest and beyond. Whether you live nearby or far away, we can help with financial counseling, schooling, housing, transportation, interpreter services and spiritual care. Read about our services for patients and families.

  • Our doctors work with other researchers to improve care and find cures for blood disorders.

    Many children with blood disorders take part in research studies along with their regular medical treatment. Your child’s doctor will talk with you about any research studies (clinical trials) that might be a match for your child. Then you can decide whether you want to try that option.

    Read more about research and clinical studies at Seattle Children’s.

Symptoms of Langerhans Cell Histiocytosis

Your child’s symptoms will depend on how Langerhans cell histiocytosis affects their body. It often damages bones, especially the skull, legs, hips, spine and arms. Not all children with the disease have the same symptoms.

These symptoms can also be caused by other health problems. See a doctor to find out the cause if your child has:

  • Pain, swelling or a 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)
  • Yellowing of the whites of the eyes and skin for some skin colors (jaundice)
  • Swollen belly from fluid collecting there
  • 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 pee more often than normal and being very thirsty
  • Delayed puberty
  • Basic signs of illness, such as fever, fatigue and weakness

Diagnosing Langerhans Cell Histiocytosis

Because Langerhans cell histiocytosis is rare, many doctors do not have experience diagnosing it. We do.

First, your doctor will:

  • Check your child for signs of illness
  • Ask about your child’s health and your family’s health history
  • Suggest tests to learn what is happening in your child’s body

Blood tests can give information about what is going on in:

  • Your child’s blood
  • The bone marrow, the soft center part of bones where blood cells are made
  • Some organs, such as the liver

If the doctor thinks that your child may have histiocytosis, a biopsy will help to confirm it. This may mean taking out a small piece of bone, lung, skin or lymph node.

Your child’s doctor may also want to take pictures of the inside of your child’s body (imaging studies). These help show areas of disease, such as damage to your child’s bones, lungs or brain.

Imaging studies may include:

  • X-ray
  • CT (computed tomography) scan
  • MRI (magnetic resonance imaging) scan

  • Langerhans cell histiocytosis is considered low risk or high risk, depending on which organs it affects.

    Low-risk disease affects 1 or more of these areas:

    • Skin
    • Bones
    • Lymph nodes
    • Pituitary gland

    High-risk disease is harder to treat. It affects 1 or more of these areas:

    • Spleen
    • Liver
    • Bone marrow

Treating Langerhans Cell Histiocytosis

Your child’s team will carefully check your child’s health to understand exactly how the condition is affecting them. Then we will tailor treatment to their needs. Sometimes the disease goes away on its own.

Sometimes children need treatments such as surgery, chemotherapy or radiation therapy.

Seattle Children’s offers these treatments for Langerhans cell histiocytosis:

  • We keep a careful watch on your child’s health so they receive the right treatment at the right time.

    Many children get checkups every 3 months. At visits, we check:

    • Any symptoms
    • Your child’s rate of growth
    Keeping track of their health in this way helps ensure your child gets the care they need before more serious problems develop. They may need to see specialists if the condition affects their:

  • Doctors may operate to remove tumors formed by extra Langerhans cells. The type of surgery your child needs depends on what part of their body is affected. Surgery is used mostly if bones are affected.

    Surgery is done at our hospital’s main campus in Seattle.

    Learn more about surgery to treat tumors at Seattle Children’s.

    In rare cases, if the liver or another organ is badly damaged, surgeons may need to remove it and transplant a healthy organ from a donor into your child.

    As the only pediatric liver transplant program in the Pacific Northwest, we provide care before, during and after liver transplant. Outcomes of patients who receive liver transplants at Seattle Children’s are among the best in the nation.

    Learn more about Seattle Children’s Liver Transplant Program.

  • Chemotherapy medicines that kill cancer cells may also help control Langerhans cells. Lower doses are given than for cancer.

    Children get some types of these medicines through a vein (called intravenous or IV). The medicine spreads around the body through the bloodstream.

    For skin problems, doctors may apply medicines right on the skin.

    Doctors use many different medicines for this disease, including prednisone, vinblastine, vincristine, etoposide (VP-16), methotrexate, cytosine-arabinoside (Ara-C), mercaptopurine (6-MP) and cladribine.

    Our patients receive chemotherapy at our main hospital campus in Seattle. Your child may get chemotherapy at our infusion center as a day procedure or stay overnight in our Cancer Care Unit.

    See more about getting chemotherapy at Seattle Children’s.

  • Radiation therapy uses high-energy particles to kill cells. A machine sends a dose of radiation into the body. Doctors aim the radiation at the place where they know or suspect there are extra Langerhans cells.

    This treatment can help control cells that are damaging a bone and making it weak. The doses are lower than those used to treat cancer.

    Learn more about Seattle Children’s Radiation Therapy Service.

  • Targeted therapy fights disease by finding a specific substance (target) and attaching to it. The target may be a protein on abnormal Langerhans cells or a change (mutation) in a gene. Different targeted therapies work in different ways. They may interfere with the cancer cells’ ability to grow, divide, make repairs or communicate with other cells.

    Some types of targeted therapy are being studied in clinical trials. Other targeted therapies may be part of standard treatment for Langerhans cell histiocytosis. Options include:

    • Tyrosine kinase inhibitors (TKIs), which help stop young blood cells from turning into abnormal Langerhans cells
    • Substances that target cells with changes in the BRAF gene and block a type of enzyme called a kinase

  • Very rarely, treatment for Langerhans cell histiocytosis includes an infusion of blood-forming (hematopoietic) stem cells from a healthy donor. Hematopoietic (him-at-oh-poy-EH-tik) stem cells are young cells that grow into blood cells. This treatment is called a stem cell transplant or bone marrow transplant.

    A transplant helps your child’s bone marrow make healthy blood cells. If this treatment may be an option for your child, we will talk with you about the risks and benefits. We also talk with you about whether a stem cell transplant fits with your family’s values, goals and priorities.

    Our Non-Malignant Transplant Program specializes in stem cell transplants for children with blood disorders and other noncancer conditions. Children need powerful drugs or radiation (called conditioning) to prepare their body for the transplant. Our team has created better ways to prepare patients and help them recover. This preparation is called reduced-intensity conditioning.

    We perform the transplants here at Seattle Children’s, working closely with Fred Hutch, our partner in the Seattle Cancer Care Alliance.

Follow-Up Care

Long-term follow-up care is important because some children have long-lasting effects from the disease. Sometimes Langerhans cell histiocytosis comes back after it has gone away.

Complications may affect:

  • Your child’s bones, liver, lung, growth, hormones, teeth or hearing.
  • The pituitary gland, causing diabetes insipidus, a condition that increases thirst and urination (peeing). It is different from diabetes mellitus, which causes high levels of blood sugar.
  • The nervous system, causing problems with speech, learning, memory, coordination or behavior.

The follow-up routine will depend on your child’s condition and their treatments.

Our Cancer Survivor Program provides long-term follow-up care to help young people stay healthy after being treated with chemotherapy or radiation in childhood, even if they didn’t have cancer.

During follow-up visits at Seattle Children’s, your child’s team will:

  • Look for any signs that disease is returning
  • Check for effects that may happen months or years after treatment ends
  • Tell you and your child about any risk for cancers that may develop as a result of being treated for Langerhans cell histiocytosis

Contact Us

Contact the Cancer and Blood Disorders Center at 206-987-2106 for an appointment, a second opinion or more information.

To make an appointment, you can call us directly or get a referral from your child’s primary care provider. We encourage you to coordinate with your pediatrician or family doctor when coming to Seattle Children’s.

Providers, see how to refer a patient.