Langerhans cell histiocytosis (LCH) factsheet
Introduction
Langerhans cell histiocytosis (LCH) is a rare condition that affects the immune system. The body makes special cells called histiocytes to fight infection.
In LCH, too many of these cells are made, which then spread and cause damage.
LCH is not cancer, but it can act like cancer. This is why it is treated by a cancer specialist.
The cause of LCH is unknown. It is not contagious but may develop after an infection or run in families. It often appears between the ages of 1 and 3.
LCH can come and go and is only life-threatening if it severely affects body parts like the:
- bone marrow
- liver
- lungs.
Signs and symptoms
LCH can cause different symptoms in each child. These symptoms can also be caused by other health conditions, which makes it difficult to diagnose.
Always see your local doctor if your child has any of the following:
- bones that damage or break easily, like the skull, legs, hips, spine and arms
- swelling or a lump in a bone that does not go away
- enlarged liver or spleen
- bulging eyes
- severe rash on the scalp or groin
- reddish-brown pinhead spots on the skin
- jaundice - yellowing of the whites of the eyes or skin
- ongoing cough and trouble breathing
- anaemia – low iron
- loss of appetite and unexplained weight loss
- ongoing fevers
- swollen belly
- diarrhoea and/or vomiting
- increased thirst
- increased urination
- ear pain
- frequent ear infections.
Diagnosis
To diagnose LCH, your child’s doctor will take a small tissue sample from the affected area to test. This is called a biopsy.
If a biopsy confirms the diagnosis, more tests will be done to check if other body parts are affected, like:
- x-rays
- blood tests
- urine tests.
Treatment
LCH sometimes goes away on its own. It will need treatment from a cancer specialist If it:
- causes pain and fever
- affects important organs
- spreads to other areas.
LCH is not cancer, but cancer treatments like chemotherapy and steroids can help.
- LCH that affects only one organ or body part is not usually life-threatening. Treatment will focus on that area to prevent damage.
- LCH that has spread and affected more organs or body parts can be more severe. Treatment will be stronger, take longer and treat the whole body.
Radiotherapy is rarely needed, as most bone issues respond well to surgery or steroids.
Management
Long-term effects of LCH
Without treatment, LCH can damage tissues and organs. Some children may have long-term effects, which is why follow-up care with a specialist is important.
Possible long-term problems include issues with the health and development of:
- bones
- liver
- lungs
- growth
- hormones
- teeth
- hearing.
It can also cause issues with:
- speech
- learning
- memory
- coordination
- behaviour.
LCH can return after it goes away. Follow-up care will depend on your child’s condition and treatment plan.
LCH and Diabetes Insipidus
Some children can develop Diabetes insipidus, a rare disorder that stops the body from being able to regulate fluid properly.
If your child has diabetes insipidus, they may need a hormone medicine called vasopressin to help the kidneys hold water. This is usually given as nose drops.
Support
It is normal for parents and carers to feel overwhelmed, anxious, or upset after an LCH diagnosis. Both practical and emotional support are important during and after treatment.
Support can be available from:
- family and friends
- health professionals
- special support services.
Speak to your child’s treatment team for more information.
