Langerhans cell histiocytosis (LCH) factsheet


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.


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:


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.


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. 


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

Cancer Institute NSW

Cancer Institute NSW

Email Send email
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

Cancer Australia

Phone1800 624 973
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

Cancer Council Helpline

Email Send email
Phone13 11 20
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

Children's Cancer Institute

Phone1800 685 686
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


Email Send email
Phone1800 226 833
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


Email Send email
Phone1800 REDKITE
Redkite provides the practical, emotional and financial support for families who have a child with cancer aged 18 or under.
Related Links
Last updated Tuesday 12th December 2023


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 2024