Langerhans Cell Histiocytosis ( LCH )
Langerhans cell histiocytosis (LCH) and hemophagocytic lymphohistiocytosis (HLH). Both conditions affect the immune system, but they do so in different ways.
Langerhans Cell Histiocytosis
Also called: LCH, eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease
What is Langerhans cell histiocytosis?
Langerhans cell histiocytosis (LCH) is a rare disorder in which the body makes too many dendritic cells. Dendritic cells are a form of histiocyte, or white blood cell. These cells play a role in the body’s immune system. They can be found in the skin, lungs, stomach, bone, eyes and intestines. In LCH, dendritic 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 most commonly affects the skin and bones but can involve any organ in the body, including lymph nodes, lungs, liver, spleen, bone marrow or brain.
What causes LCH?
The exact cause of LCH in children is unknown. In the past, researchers thought infections or factors in the environment could trigger the disease. Newer research shows that LCH arises when mutations (changes) develop in genes that control how dendritic cells behave.
How common is LCH?
About one out of every 200,000 children develop Langerhans cell histiocytosis each year.
LCH can be found in children from birth through adulthood. It is most often found in children ages 1 to 3.
What are the symptoms of LCH?
Symptoms of LCH can vary depending on the part of the body that is affected. Some common symptoms include:
- Skin
- Rashes that may be 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 or feet, ribs, pelvis and spine
- Stomach problems
- Pain in the abdomen or pelvis
- Yellow skin
- Vomiting
- Diarrhea
- Blood in stool
- Liver/spleen
- Enlarged liver or belly
- Abnormal blood tests
- Hormones (endocrine system)
- Increased thirst or dehydration
- Increased need to urinate
- Fatigue
- 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
- Blurred or loss of vision
- Bulging eyeballs
- Problems swallowing or speaking
- Mouth
- Mouth ulcers
- Swollen or bleeding gums
- Swollen lymph nodes in neck
- Ears
- Loss of hearing
- Discharge from ear canal
- Redness
- Cysts
How is LCH diagnosed?
If your child has symptoms of LCH, a doctor will run tests before diagnosis. Depending on the symptoms, the doctor may perform a few of the following tests:
- Physical exam (including neurological testing)—The doctor will measure your child’s height, weight and temperature. You may also be asked about your family’s medical history, which can include any symptoms and past illnesses. Neurological exams may include studying your child’s ability to walk, coordination and memory.
- Complete blood count (CBC)—A small sample of blood is taken from the child’s vein. The different types of blood cells in the sample are then counted in the lab. Children with LCH in their bone marrow may have decreased cell counts in their blood.
- Blood chemistry test—Blood chemistry tests measure the amount of certain substances (such as electrolytes, sugar, enzymes, fats and proteins) in the body. Analysis of the blood chemistry tests may help identify if there is LCH in the liver, kidneys or other organs.
- Bone marrow aspiration and biopsy—The child is usually given anesthesia, a medicine that causes sleep, for this test. 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—This test may occur if your child is having symptoms that relate to the stomach or digestive tract. A tube will be inserted in your child’s mouth or rectum that the doctor can use to look at the tissue in the esophagus, stomach and small intestine.
Additionally, your child’s doctor may use X-rays, CT scans, PET scans or MRI machines to take detailed photos of the inside of your child’s body. These photos can provide a clearer idea of any abnormalities.
How is LCH treated?
Treatment for LCH is based upon each individual child. Children with LCH can be either low-risk (less-intensive) or high-risk (more extensive) based on the extent of the disease and which body parts are affected.
Low-risk LCH typically involves the skin, bones, lymph nodes and brain. Low-risk LCH may involve multiple organs, but will generally not affect liver, bone marrow or spleen. It is possible that some children with low-risk LCH will improve without therapy.
If just a single bone is affected, surgery is usually the first step. The surgery is used to confirm the diagnosis and may remove the dendritic-cell growth. Doctors watch the child carefully afterward to check for a possible return of LCH.
High-risk LCH generally involves multiple organs including the liver, bone marrow or spleen. For children with high-risk LCH, the best treatment is chemotherapy (chemo) that lasts about a year.
- Chemotherapy (“chemo”)—uses powerful medicines to kill cells or stop them from growing (dividing) and making more cells.
- Chemo may be injected into the bloodstream, so that it can travel throughout the body.
- Some chemo may be given by mouth.
- Combination therapy uses more than one type of chemo at a time.
What are the survival rates for Langerhans cell histiocytosis?
- About 99% of children with low-risk LCH survive.
- About 80% of children with high-risk LCH survive.
