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.

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:

• x-rays
• blood tests
• urine tests.

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.