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Langerhans Cell Histiocytosis Treatment

Also called: LCH, eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease

Langerhans cell histiocytosis (LCH) is a rare disorder in which the body makes too many Langerhans cells. Langerhans cells are a type of white blood cell. They play a role in the body’s immune system. Langerhans cells can be found in the skin, lungs, stomach, bones, eyes, and intestines. In LCH, these cells build up, forming tumors and disrupting the normal function of tissues.

LCH is a form of cancer and often, but not always, requires treatment with chemotherapy.

LCH often affects the skin and bones. But it can involve any organ in the body, including lymph nodes, lungs, liver, spleen, bone marrow, or brain.

Langerhans cell histiocytosis symptoms

Symptoms of LCH can vary depending on the part of the body that is affected. Symptoms may include:

  • Skin
    • Rashes that are scaly or waxy
    • Hair loss
    • Crusty or oily patches on the scalp (may be misdiagnosed as cradle cap)
  • Bone pain
    • Lumps or lesions on the skull, upper or lower limbs, hands, feet, ribs, pelvis, and spine
  • Stomach problems
    • Pain in the abdomen (belly) or pelvis
    • Yellow skin
    • Vomiting
    • Diarrhea
    • Blood in stool
  • Liver or spleen
    • Enlarged liver or belly
    • Abnormal blood tests
  • Hormones (endocrine system)
    • Increased thirst or dehydration
    • Increased need to urinate (pee)
    • Feeling tired
    • Rapid weight change (either up or down)
    • Fever or sweats
    • Change in menstrual cycle
  • Lungs
    • Chest pain
    • Problems breathing or shortness of breath
    • Cough
  • Brain or central nervous system
    • Headaches
    • Dizziness
    • Seizures
    • Blurry vision or vision loss
    • Bulging eyeballs
    • Problems swallowing or speaking
    • Decreased coordination
  • Mouth
    • Mouth ulcers
    • Swollen or bleeding gums
    • Swollen lymph nodes in neck
  • Ears
    • Hearing loss
    • Discharge from ear canal
    • Redness
    • Cysts

Langerhans cell histiocytosis causes

In the past, scientists thought infections or factors in the environment could trigger the disease. New research shows that LCH is caused by changes (mutations) in BRAF and other genes that control cell growth.

About 1 out of every 200,000 children develop LCH each year. LCH can be found in children from birth through adulthood. It is most often found in children 1 to 3 years old.

Langerhans cell histiocytosis diagnosis

To diagnosis LCH, your health care provider will perform a medical exam and ask about your family history. They will do several types of tests based on your child’s symptoms.

Tests may include:

  • Complete blood count (CBC): A small blood sample is taken from a vein. The different types of blood cells in the sample are then counted in the lab. Children with LCH may have decreased cell counts in their blood.
  • Biopsy: A procedure to collect abnormal tissue to be analyzed by a pathologist and confirm the diagnosis.
  • Blood chemistry test: Tests to measure the amount of certain substances in the blood. These tests can help detect LCH in the liver, kidneys, or other organs.
  • BRAF gene testing: A lab test to detect changes (mutations) to the BRAF gene.
  • Immunohistochemistry: Used to help diagnose cancer.
  • Bone marrow aspiration and biopsy: A thin, hollow needle is put into a bone, often in the hip. A small amount of bone marrow is aspirated (taken out). For the biopsy, a slightly larger needle is used to take out a small piece of bone. Experts look at the bone and the bone marrow in the lab.
  • Endoscopy: Used to look at the tissue in the esophagus, stomach, and small intestine.
  • Imaging tests: Scans and imagining tests such as x-rays, ultrasound, CT scans, PET scans or MRI scans take detailed pictures of the inside the body.
  • Neurological exams: Tests may include measuring different aspects of muscle, nerve, and brain function.
  • Urinalysis: A urine sample is used to check urine contents such as glucose, protein, and blood cells.
  • Pulmonary function test (PFT): A breathing test to see how well the lungs work.

Types of Langerhans cell histiocytosis

LCH can be either low risk or high risk based on the extent of the disease and which body parts are affected.

Low-risk LCH typically involves the skin, bones, and lymph nodes. It may also involve multiple organs. Low-risk LCH does not affect the liver, bone marrow, or spleen.

High-risk LCH involves multiple organs including the liver, bone marrow, and/or spleen.

Langerhans cell histiocytosis treatment

Treatment for LCH is based on risk level. Some children with low-risk LCH may improve without treatment.

If just a single bone is affected, biopsy by a surgeon or interventional radiologist is usually the first step. The surgery is used to confirm the diagnosis and remove some or all of the cell growth. The care team will continue to monitor your child to see if LCH comes back.

The best treatment for high-risk LCH is usually chemotherapy (chemo). It may last about a year. Chemotherapy uses powerful medicines to kill cells or stop them from growing (dividing) and making more cells. Newer treatments that target cells with mutations in the BRAF gene or other related genes are currently being investigated.

Prognosis for Langerhans cell histiocytosis

The overall survival rate for low-risk LCH is about 99%. For children with high-risk LCH, the survival rate is about 85%.

Langerhans cell histiocytosis care at St. Jude

  • St. Jude researchers are looking for ways to improve the outcome for children with high-risk disease, disease that does not respond to initial therapy, or disease that recurs.
  • Your child will have access to a care team of specialists to plan the best treatment for your child’s needs. Care team members may include: doctors and nurses who treat this condition; surgeons; doctors who specialize in radiation therapy (radiologists), in hormones (endocrinologists), and in diagnosis (pathologists); experts in diagnostic imaging and nuclear medicine; genetic counselors; dietitians; speech therapists; physical therapists; occupational therapists; child life specialists; psychologists; Quality of Life team members; and others.
  • The medical team works closely with scientists to develop new treatments for LCH.
  • St. Jude is the only National Cancer Institute–designated Comprehensive Cancer Center just for children. A Comprehensive Cancer Center meets rigorous standards for research that develops new and better approaches to prevent, diagnose, and treat cancer.
  • The nurse-to-patient ratio at St. Jude is unmatched. It is about 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit. 

Langerhans cell histiocytosis clinical trials

St. Jude offers clinical trials and cancer research studies for children, teens, and young adults for Langerhans cell histiocytosis. Learn more about clinical research at St. Jude

Recruiting
LCH-IV: Affects of Tailored Treatments for Langerhans Cell Histiocytosis

Study goal:

The main goal of this study is to see how tailored treatments affect LCH in children and adolescents.

Age:

Younger than 18 years old

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Seeking treatment at St. Jude

Patients accepted to St. Jude must have a disease we treat and must be referred by a physician or other qualified medical professional. We accept most patients based on their ability to enroll in an open clinical trial.

How to seek treatment

Contact the Physician / Patient Referral Office

Submit online referral form

Call: 1-888-226-4343 (toll-free) or 901-595-4055 (local)  | Fax: 901-595-4011 | Email: referralinfo@stjude.org | 24-hour pager: 1-800-349-4334

 

Learn more