Introduction
Langerhans cell histiocytosis (LCH) is a rare disorder that involves the immune system. The body makes cells called histiocytes which help to fight infection in the body. When the body makes too many of these cells, they can move around the body and cause damage.
LCH is not a cancer, but it can behave like one. This is why it is treated by a cancer specialist.
LCH may show up between the ages of 1 and 3 years old. It is not currently known what causes LCH to happen.
Signs and symptoms
Each type of LCH presents with different symptoms and has different outcomes, so it can be difficult to know what the signs and symptoms are.
Below is a list of signs and symptoms that can be caused by LCH but could also be cause by another health issue. If your child shows any of these symptoms, it is important to take them to your local doctor to be checked.
- bones that damage or break easily, for example the skull, legs, hips, spine and arms
- swelling or a lump in a bone that does not go away
- enlarged liver or spleen
- issues with the eyes, like bulging
- severe scalp and/or groin rash
- reddish-brown pinhead spots on the body
- jaundice - yellowing of the whites of the eyes or skin
- persistent cough and trouble breathing
- anaemia – iron deficiency
- loss of appetite and unexplained weight loss
- recurrent fevers
- swollen belly
- diarrhoea - loose and watery poo
- vomiting
- increased thirst and need to urinate or wee
- ear pain or recurring ear infections.
Diagnosis
To diagnose LCH, your child’s doctor will remove a small section of the affected area and perform tests to look for signs of disease. This is called a biopsy.
One the biopsy results confirm the diagnosis, the doctor will do some more tests to figure out which other body parts or organs are affected by the LCH. Tests can include:
- x-rays
- blood tests
- urine tests.
Treatment
In some cases, LCH will suddenly heal on its own. In other cases where there is pain, fever, weight gain or important organs are affected, treatment is managed by an oncologist (cancer specialist). LCH is not cancer, but chemotherapy and steroids can work well to treat it.
If the LCH is only affecting one organ, the treatment will be given locally. This means treatment is delivered just to that specific organ.
If the LCH is affecting a range of different organs or body parts, it will be treated generally. This means treatment is delivered to the whole body.
Radiotherapy is rarely needed for LCH because most bone lesions or damage will respond to surgery or steroids.
For children who also have a condition called diabetes insipidus, a hormone called vasopressin may be needed to help keep water in the kidneys. This is usually in the form of nose drops. Diabetes insipidus is a rare disorder that stops the body from being able to regulate fluid properly.
Management
Cause of Langerhans cell histiocytosis
The cause of LCH is unknown. LCH is not infectious, but it may develop after an infection or may be passed down in some families.
LCH can come and go spontaneously and is only life threatening if important organs are severely affected, including:
- bone marrow
- liver
- lungs.
Tissue and organ damage from Langerhans cell histiocytosis
If LCH is left untreated, it can cause damage to tissues and organs all over the body. Long-term follow-up care with your child’s specialist doctor is important because some children have long-lasting effects from the disease, including:
- issues with the development or health of:
- bones
- liver
- lungs
- growth
- hormones
- teeth
- hearing.
- problems with:
- speech
- learning
- memory
- coordination
- behaviour.
- development of diabetes insipidus - a condition that stops the body from being able to regulate fluid properly
Sometimes LCH can come back after it has gone away.
Follow-up care will depend on your child’s condition and their treatments.
Effectiveness of treatment
If LCH is only in one part of the body, it is usually not life threatening, and the main aim of treatment is to prevent damage to the organ that is affected.
If the LCH has spread and is affected many organs or body parts, there is a greater risk of severe illness. Treatment is usually longer and more aggressive.
Generally, the younger the child and the more organs involved, the stronger the disease is, which means more intense treatment is required.
Support
Parents and carers can often feel overwhelmed, scared, anxious and upset after a diagnosis of LCH. These are all normal feelings. Having practical and emotional support during and after diagnosis and treatment for LCH is important. Support may be available from family and friends, health professionals or special support services.
Resources and more information
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Cancer Institute NSW
The Institute's website provides information, support, research and data on cancer.
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Cancer Australia
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Cancer Council Helpline
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Children's Cancer Institute
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Canteen
