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This information is about Langerhans' cell histiocytosis (LCH) in children. It may be helpful to read it alongside our general information on children's cancer, which contains detailed information about cancers and cancer-like conditions in children, their diagnosis and treatment, and the support services available.
About 50 children in the UK develop Langerhans' cell histiocytosis (LCH) each year. It can affect children of any age, and is more common in boys than girls.
LCH is not strictly a cancer but, rather, a cancer-like condition that may be treated with chemotherapy. In its more serious forms LCH can behave like a cancer and is 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 which are part of the immune system and are found in many parts of the body. There are two types of histiocyte: macrophage monocyte cells which destroy harmful proteins, viruses and bacteria in the body; and dendritic cells which 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 spread via the bloodstream into many 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:
The cause of LCH is unknown. It cannot be caught from other people and it is not passed on in families.
The symptoms of LCH will depend upon which part of the body is affected, and whether the disease is affecting more than one part of the body.
If LCH affects the bone, this can cause pain in the bone and/or swelling/lumps on the skull. If the skin is affected, a skin rash, such as cradle cap and nappy rash, may occur. A discharge from the ear or hearing problems can occur if the ear is affected. If LCH affects the lungs or chest, the child may have breathing difficulties. LCH within the abdomen may cause tummy problems (diarrhoea) and liver problems, including possible jaundice. The lymph glands may be enlarged. Children may be irritable and have a poor appetite.
In 2–3 out of every 10 children with multi-system disease, 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 again can 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 to decide if the disease is a single-system or multi-system type. Tests are likely to include the removal of a sample of cells (biopsy), and this is usually done in an operation, under a general anaesthetic. The cells are examined under a microscope. An MRI (magnetic resonance imaging) scan of the brain may also be carried out.
Any tests and investigations that your child needs will be explained to you. The general information on children's cancers gives details of what the tests and scans involve.
Single-system LCH may disappear on its own without any treatment. This may occur following a biopsy. In some children, treatment such as surgery and corticosteroids (for example prednisolone) may be used.
Multi-system disease 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.
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 upon the particular treatment being used and the part of the body that is being treated.
Side effects of chemotherapy can include: feeling sick (nausea) and vomiting; hair loss; an increased risk of bruising and bleeding; 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 are reversible when treatment stops.
Some children may develop after effects of the disease. 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 the treatment they receive.
Possible late 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 of the standard treatment). 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; hospitals that carry out trials do however have access to good equipment and 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. Your child's medical team will talk to you about taking part in a clinical trial (if appropriate) 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 if it is right for your child.
Between eight and nine of every 10 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 is 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.
Your child may have a range of powerful emotions throughout their experience of illness. Our booklet, Peppermint Ward, is a storybook for younger children with cancer. It looks at the issues they and their family may face, and helps them to explore their feelings. You can order the booklet online.
This section has been compiled using information from a number of reliable sources, including:
For further references, please see the general bibliography.
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