• Langerhans Cell Histiocytosis Overview

    Langerhans cell histiocytosis (LCH) is a rare disorder that occurs when a child has too many of a certain type of white blood cell called Langerhans cells. These cells normally reside in the skin and help fight infection and destroy certain foreign substances in the body.  LCH can impact in one organ or body system (unifocal) or more than one part of an organ or body system (multifocal). It can also affect multiple organs and body systems.

    Langerhans Cell Histiocytosis Treatment at Dana-Farber/Boston Children’s

    Children and young adults with Langerhans cell histiocytosis are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center in Boston, MA, through our Histiocytosis Program. Continue reading to learn more about LCH or visit the Histiocytosis Program homepage to learn about our expertise.

    Symptoms & Diagnosis

    In LCH, the Langerhans cells accumulate in bone and other parts of the body, particularly the head and neck, and can cause a variety of symptoms. They can also be found in the ribs, sternum, long bones of the arm and leg, vertebra of the spine, and the pelvis.  The most common symptoms include:

    • Skin rash
    • Tenderness or pain originating from a bone
    • Loose or lost teeth
    • Swollen gums
    • Multiple ear infections
    • Eyelid swelling or other vision problems
    • Excessive thirst and urination
    • Fever and night sweats
    • Weakness
    • Failure to gain weight

    The most common age at diagnosis is two or three years, but children can be diagnosed at birth and into their teenage years. The most conclusive diagnostic procedure for LCH is a biopsy. Other tests include:

    • X-ray
    • Magnetic resonance imaging (MRI)
    • Computed tomography (CT or CAT) scan
    • Liver or bone marrow biopsy – sample tissues from these areas can help determine how much of the body is affected by LCH
    • Bone scan – this test does not distinguish between a tumor, infection or fracture, but can indicate abnormalities in bones
    After all tests are completed, doctors will be able to outline the best treatment options.
     

    Treatment & Care Options

    Treatment for LCH varies widely from child to child and, in some cases, is similar to that of cancer (though LCH is not a type of cancer). Oftentimes, the disease goes away on its own. Otherwise, it may be treated with:

    • Medications – including steroids (prednisone), nonsteroidal anti-inflammatories (indomethacin) and bisphosphonates (zoledronate), which can lessen bone pain
    • Surgery – removal of growths of LCH cells
    • Radiation therapy – small doses of radiation can help stop the growth of Langerhans cells in specific areas of the body
    • Chemotherapy 

    Progressive or Recurrent Disease

    LCH may recur in the same place or in other parts of the body, most often in bone, the ears, skin or pituitary gland. The disease often recurs the year after stopping treatment, but there have been reports of recurrence up to five years after the initial disappearance of the disease.

     

    Long-term Outlook

    The prognosis is excellent for children with single-system disease, with a 99 percent survival rate. Children with multi-system disease have a 66 percent survival rate. Complications occur in about 35 percent of patients, the most common being orthopedic disabilities, hearing impairment, diabetes insipidus and skin scarring. Less common complications include chronic pulmonary dysfunction, liver cirrhosis, secondary malignancies such as acute lymphoblastic leukemia or solid tumors, and growth retardation.
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