Langerhans' cell histiocytosis (LCH) in children
This information is about a condition called Langerhans' cell histiocytosis (LCH) in children. LCH is not strictly a cancer, but it's a cancer-like condition that may be treated with chemotherapy.
More children than ever are surviving childhood cancer. There are new and better drugs and treatments. But it remains devastating to hear that your child has cancer, and at times it can feel overwhelming. There are many healthcare professionals and support organisations to help you through this difficult time.
Understanding more about the cancer your child has and the treatments that may be used can often help parents to cope. We hope you find the information here helpful. Your child's specialist will give you more detailed information. If you have any questions it’s important to ask the specialist doctor or nurse who knows your child’s individual situation.
Langerhans' cell histiocytosis (LCH)
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LCH is not strictly a cancer, but it's a cancer-like condition that may be treated with chemotherapy. More serious forms of LCH can behave like cancer and are therefore usually treated by children's cancer specialists (paediatric oncologists).
Langerhans' refers to Dr Paul Langerhans, who first described the cells in the skin from which LCH develops.
Histiocytes are cells that are part of the immune system, and are found in many parts of the body. There are two types of histiocytes:
macrophage/monocyte cells - these destroy harmful proteins, viruses and bacteria in the body
dendritic cells - these stimulate the immune system.
Langerhans' cells are dendritic cells and are normally only found in the skin and major airways. In LCH, the Langerhans' cells are abnormal and may be found in different parts of the body, including the bone marrow, skin, lungs, liver, lymph glands, spleen and pituitary gland. When Langerhans' cells are present in these tissues, they may cause damage.
LCH is divided into two groups:
single-system LCH - when the disease affects only one part of the body, for example the skin or the bone
multi-system LCH - when it affects more than one part of the body.
About 50 children in the UK develop LCH each year. It can affect children of any age, and is more common in boys than in girls.
The cause of LCH is unknown. It cannot be caught from other people and is not passed on in families.
Signs and symptoms of LCH
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The symptoms of LCH will depend on which part of the body is affected and whether the disease is affecting more than one part of the body.
The lymph glands may be enlarged, and the child may be irritable and have a poor appetite.
Pain in the bone and/or swelling and lumps on the skull can occur if LCH is affecting the bone.
A skin rash such as cradle cap or nappy rash may occur if the skin is affected.
A discharge from the ear or hearing problems can occur if the ear is affected.
The child may have breathing difficulties if LCH affects the lungs or chest.
Tummy problems such as diarrhoea and liver problems including jaundice can occur if LCH is within the abdomen.
In 2-3 out of every 10 children with multi-system disease (20-30%), the pituitary gland at the base of the brain is affected, causing hormonal problems. This can lead to the child passing larger amounts of urine and being very thirsty. This is called diabetes insipidus, which is different from sugar diabetes and can be well-controlled with specific medicines. Occasionally, other pituitary hormones may be affected, causing poor growth or delayed puberty, which can also be treated.
A variety of tests and investigations may be needed to diagnose LCH. X-rays will often be taken of the bones, the skull and the lungs. Blood tests will also be taken. These tests help the doctors decide whether the disease is a single-system or multi system type.
Tests are likely to include the removal of a sample of cells (a biopsy), which is usually done in an operation under a general anaesthetic. The cells are then examined under a microscope. An MRI (magnetic resonance imaging) scan of the brain may also be carried out. When your child is having the tests, they may need to stay in hospital.
Any tests and investigations that your child needs will be explained to you. We have more information about children’s cancers, including details of what the tests and scans involve.
Single-system LCH may disappear on its own without any treatment. In some children, treatment such as surgery and corticosteroids (such as prednisolone) may be used.
Multi-system LCH is usually treated with chemotherapy and corticosteroids. The length of treatment varies from child to child.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells, and corticosteroids are hormonal substances that are naturally produced in the body. Both chemotherapy and corticosteroids can help to destroy the LCH cells.
Side effects of treatment for LCH
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Chemotherapy and corticosteroids used to treat LCH may cause side effects, and your child's doctor will discuss these with you before treatment starts. Any possible side effects will depend on the particular treatment being used and the part of the body that’s being treated.
Side effects of chemotherapy can include feeling sick (nausea) and vomiting, hair loss, an increased risk of bruising and bleeding, and tiredness. Corticosteroids may cause a rounding of the face (sometimes called a moon face), mood changes and an increased appetite, which can lead to weight gain. All of these side effects should get better when treatment stops.
Late side effects
Some children may develop after-effects (or late effects) after LCH. These effects can happen within months or years of the diagnosis and are more likely to be due to the disease itself than to the treatment given. However, a few children may develop late effects from the drugs or treatment they receive.
Possible late side effects include reduction in bone growth, delayed puberty, hearing problems and lung problems.
Many children have their treatment as part of a clinical research trial. Trials aim to improve our understanding of the best way to treat an illness, usually by comparing the standard treatment with a new or modified version. Research has found that people receiving treatment within clinical trials tend to do as well as, or better than, similar patients treated outside clinical trials.
This does not mean that trial treatments are always better. However, hospitals that carry out trials do have access to good equipment and, like all hospitals, they follow precise guidelines when giving treatment.
The treatment trials for LCH are set up and organised by The Histiocyte Society, a group of international specialists in histiocytosis. If appropriate, your child's medical team will talk to you about taking part in a clinical trial and will answer any questions you may have. Written information is often provided to help explain things.
Taking part in a research trial is completely voluntary, and you'll be given time to decide whether it's right for your child.
8-9 out of every 10 (80-90% of) children who develop LCH will recover from it. Sometimes the disease can come back, and so children will have regular check-ups in the outpatients clinic.
Follow-up is also important to look out for late side effects. If you have specific concerns about your child’s condition and treatment, it's best to discuss them with your child’s specialist medical team who know the situation in detail.
As a parent, the fact that your child has a serious illness is one of the worst situations that you can be faced with. You may have many different emotions, such as fear, guilt, sadness, anger and uncertainty. These are all normal reactions and are part of the process that many parents go through at such a difficult time.
Our section on children’s cancers talks about the emotional impact of caring for an unwell child and suggests sources of help and support. It also discusses the range of powerful emotions your child may experience throughout their illness, including how you can support your child.
We also have a booklet called Katie's Garden, which is a storybook for primary school-age children about a girl's experience of cancer.
Children's Cancer and Leukaemia Group (CCLG)
CCLG coordinates research and care for children and their parents. There are 21 CCLG specialist centres for the treatment of childhood cancer and leukaemia, covering all areas of the UK and Ireland (there's a map of the centres on the website).
CLIC Sargent provides clinical, practical, financial and emotional support to children with cancer.
The Histiocytosis Association
The Histiocytosis Association is an international partnership of patients, families, physicians and friends, which aims to promote scientific research and education related to histiocytosis. It aims to provide solutions to some of the problems that are specific to patients suffering from this disease, and to offer support to patients and their families.
Histiocytosis Research Trust
The Histiocytosis Research Trust was formed to promote and fund scientific research into histiocytoses. The trust has also created a ‘Circle of Friends’ for mutual support through information, newsletters and roadshows led by medical experts. They are in touch with many families around the UK and overseas.
This section has been compiled using information from a number of reliable sources, including:
Voute PA, et al. Cancer in Children: Clinical Management. 5th edition. 2005. Oxford University Press.
Pinkerton, et al. Evidence-based paediatric oncology. 2nd edition. 2007. Blackwell Publishing.
McLain KL. Langerhans cell histiocytosis, Juvenile xanthogranuloma, and Erdheim-Chester disease. UpToDate. www.uptodate.com (accessed September 2012).
Thanks to Dr Kevin Windebank, Senior Lecturer in Child Health and Consultant Paediatric and Adolescent Oncologist, and all of the people affected by cancer who reviewed this information.
We worked with the Children's Cancer and Leukaemia Group (CCLG) to write our information about children's cancer.