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.

In some children with the disease, extra cells build up and damage 1 area or organ in their bodies. In other children, extra cells build up in many places. Langerhans cell histiocytosis is most likely to harm 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. Although this condition is not cancer, doctors treat it the same ways they treat cancer.

  • 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.

Langerhans Cell Histiocytosis at Seattle Children’s

USNWR BadgeThis is a rare disease, and most doctors do not 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.

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

If you would like an appointment, ask your primary care provider to refer you. If you have a referral or would like a second opinion, contact the center at 206-987-2106.

Providers, see how to refer a patient.

  • Our doctors are nationally known for treating children who have blood disorders, including Langerhans cell histiocytosis. The doctors who will 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
    • Stem cell transplant, in rare cases

    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
    • 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. U.S. News & World Report consistently ranks Seattle Children’s as the top pediatric facility in the Northwest and among the nation’s best children’s hospitals.

    Our physician-scientists help set national standards for the care of young people with blood disorders. We provide the most advanced treatments in our region.

    Children do not react to illness, injury, pain and medicine in the same way as adults. They need – and deserve – care designed just for them. We plan your child’s treatment based 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 take positive steps right away by offering same-day appointments for children with urgent needs. If needs are not urgent, new appointments can be made within 1 week.

    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.

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 or skin (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
  • 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, the doctor will:

  • Check your child for signs of illness
  • Ask about your child’s health and your family’s health history

The doctor may suggest tests to learn what is happening in your child’s body.

  • Blood tests give information about your child’s blood, bone marrow and organs such as the liver.
  • A biopsy of a piece of bone, lung, skin or lymph node helps confirm the diagnosis.
  • Imaging studies look at areas of disease, such as damage to your child’s bones, lungs or brain.
  • 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.

  • 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.

    Learn more about surgery to treat tumors at Seattle Children’s. Surgery is done at our hospital campus in Seattle. 

    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.

    We have the only pediatric liver transplant program in the Pacific Northwest, with excellent outcomes. We care for your child before, during and after liver transplant.  

    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. Doctors use many different medicines for this disease.

    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 (topical treatments).

    Our patients receive chemotherapy at our hospital campus in Seattle. Your child may get chemotherapy at our outpatient infusion unit 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 beams 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 affect 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 stem cells from a healthy donor. This is called a stem cell transplant or bone marrow transplant or hematopoietic (him-at-oh-poy-EH-tik) cell 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
    • 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 non-cancer conditions. Children need powerful drugs or radiation (called conditioning) to prepare their body for the transplant. Our team has created better ways to prepare children and help them recover. This preparation is called reduced-intensity conditioning. We keep improving the conditioning treatments to increase survival and reduce complications.

    We perform the transplants here at Seattle Children’s, working closely with Fred Hutch, our partner in the Seattle Cancer Care Alliance. Fred Hutch pioneered this lifesaving procedure and is one of the largest stem cell transplant centers in the world.

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, lungs, 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.

Follow-up care 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 

If you would like an appointment, ask your child’s primary care provider to refer you.

If you have a referral or would like a second opinion, call the Cancer and Blood Disorders Center at 206-987-2106 or by email.

Providers, see how to refer a patient.

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