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:

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. 

Resources and more information

Cancer Institute NSW

title
Cancer Institute NSW

Email Send email
Biography
NSW's cancer control agency, established to lessen the impact of cancer across the State.

The Institute's website provides information, support, research and data on cancer.
Related Links
Cancer Australia

title
Cancer Australia

Phone1800 624 973
Biography
Cancer Australia was established by the Australian Government in 2006 to benefit all Australians affected by cancer, and their families and carers. Their website provides resources and information regarding cancer.
Related Links
Cancer Council

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Cancer Council Helpline

Email Send email
Phone13 11 20
Biography
Anyone can call this helpline - cancer patients, people living with cancer, their families, carers and friends. Specially trained staff are available to answer questions about cancer and offer emotional or practical support.
Related Links
Children's Cancer Institute

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Children's Cancer Institute

Phone1800 685 686
Biography
CCI is an Australian medical research institute wholly dedicated to curing childhood cancer. Their website offers information on childhood cancer and opportunities to volunteer, fundraise or donate to help support their work.
Related Links
Canteen

title
Canteen

Email Send email
Phone1800 226 833
Biography
Canteen supports 12-25 year-olds dealing with cancer diagnosis, a close family member’s cancer or the death of a loved one. Their services also now extend to parents, because when they cope better with cancer their children experience less distress.
Related Links
Redkite - For families facing childhood cancer

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Redkite

Email Send email
Phone1800 REDKITE
Biography
Redkite provides the practical, emotional and financial support for families who have a child with cancer aged 18 or under.
Related Links

Disclaimer

This factsheet is provided for general information only. It does not constitute health advice and should not be used to diagnose or treat any health condition.

Please consult with your doctor or other health professional to make sure this information is right for you and/or your child.

The Sydney Children’s Hospitals Network does not accept responsibility for inaccuracies or omissions, the interpretation of the information, or for success or appropriateness of any treatment described in the factsheet.

© Sydney Children’s Hospitals Network 2025


This factsheet was produced with support from John Hunter Children's Hospital.