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 Langerhans Cell Histiocytosis (LCH) in children;

(previously known as: Eosinophilic Granuloma, Letter-Siwe Disease, Hand Schuller-Christian Syndrome, and Histiocytosis X)


How Common is Langerhans Cell Histiocytosis in children:

The British Paediatric Surveillance Unit (BPSU) recorded 50 new cases of LCH in children, within the UK and Eire in 2007. For more information on this study, please go to bpsu.inopsu.com   

There have been no studies on the incidence of LCH in adults, so the number of adults affected each year has yet to be confirmed. Despite the rarity of LCH in adults, the Histiocytosis Association of America estimates that 1 in 560,000 adults may be affected by this disease. Please go to www.histio.org for further information.

What is Langerhans Cell Histiocytosis (LCH) 

LCH is a cancer-like illness that can be seen in children and adults. In cancer there is a genetic mutation, this has not been found in LCH. This disease is part of a group of syndromes called histiocytoses, which are characterised by an abnormal proliferation (too many) of histiocytes (dendritic cells and macrophages). Histiocytes are derived from the stem cell in the bone marrow . They are white blood cells that are part of our immune system and help us fight infections.

The cause of LCH is unknown.  It may be triggered by an unusual reaction of the immune system from something commonly found in the environment, reactive disease. It is not a known infection and it is not known to be hereditary or contagious. In cancer a genetic mutation occurs, in LCH  genetic mutation has not been discovered. LCH can behave like cancer and is usually treated by oncologists.

Langerhans cells are not counted in a normal blood count as they are usually found in tissues rather than the blood: like skin, lymph nodes, spleen or lungs. 

At present, dendritic cells are being used in new cancer treatment trials. Maybe, if scientists knew more about LCH this could hold the key to understanding other 'true' cancers and lead to better treatments and ultimately a cure.

WHY DO WE HAVE LANGERHANS CELLS? Fundamentally, our skin has two parts, the outer layer – epidermis, and the inner layer – dermis. Langerhan’s Cells live in between these two layers and their purpose is to protect our bodies from foreign bodies. They stay in between the layers of skin until something stimulates them, such as an infection, bacteria or another invader, such as cancer. They play a critical role in skin immune surveillance, although they represent no more than 2% of all epidermal cells. They are predominantly found in the skin, lymph nodes, spleen and lungs. If they are discovered in your liver or bone marrow they are distinctly abnormal.

In a healthy body, Langerhan’s cells form a network in the skin and take up foreign materials such as bacteria or viruses that break through the skin’s surface. The Langerhan’s cells then migrate to lymph nodes to start a protective immune response. Fundamentally they act as carriers, taking bacteria (invaders) to the lymph nodes, so they can be destroyed.

HOW WILL A DOCTOR CONFIRM YOU HAVE LANGERHANS CELL HISTIOCYTOSIS:  Due to its rarity, sometimes LCH is difficult to diagnose and very often missed or mistaken as a true malignancy. Some of you reading this may have had several visits to hospital before a doctor even suspected LCH.To confirm LCH you will need a biopsy. Once a diagnosis has been made various test will be performed to establish the extent of the disease; this could include - blood tests, MRI Scan, Bone Marrow Aspirate, Bone Scan and/or x rays. Your consultant will discuss everything with you.

LCH PROTOCOLS –

 

Due to the rarity of LCH, it takes a long time to gather enough data and establish the best treatment/length of treatment for LCH. For more information on Protocols by The Histiocyte Society, please click here .

The Histiocyte Society is a nonprofit organization, comprised of an international group of more than 200 physicians and scientists, committed to improving the lives of patients with histiocytosis by conducting clinical and laboratory research into the causes and treatment of this disease. The importance of Clinical trials in the fight against Histiocytosis, by Milen Minkov, M.D.; James Whitlock, M.D. article click here

Approximately 15% of children with LCH will die , depending on the extent of the disease and sites affected.

STAGES OF LCH

LCH is divided into two groups:

  • single-system LCH, when the disease affects only one part of the body, for example, the skin or the bone
  • multi-system LCH, when it affects more than one part of the body

single system disease can be classed as 'unifocal' - one area, of one part of the body is affected.; 'Multiocal' if there is more than one lesion/tumour in the bones. For example, if your child has a tumour/lesion in his pelvis and skull, this is classed as single system, multifocal LCH.

TREATMENT: Treatment will vary depending on the sites affected. Some patients with limited involvement require no treatment and will go into spontaneous remission. Others will require chemotherapy, steroids, radiotherapy and in some cases a bone marrow transplant. With your permission, your child will be treated according to the latest treatment protocol. LCH Protocol IV will be the next protocol.

Single system LCH may disappear on its own without any treatment. This may occur following a biopsy. Some children may have a curettage and corticosteroids, like prednisolone. For more informaton on a curettage click here

Mulitifocal or multisystem disease is normally treated with chemotherapy and steroids.

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SYMPTOMS OF LANGERHANS CELL HISTIOCYTOSIS:The most common presentation of LCH is a rash or a painful lump in the bone. Other symptoms include;

Jaundice -  If liver or spleen is affected

 (if you have any photos of LCH involvement of the skin, please contact us at info@histiouk.)
Skin involovement is seen in over 1/3 of children with LCH. The most commonly affected sites are the scalp, neck and stomach. This can be part of mulitsytem disease or the skin can be affected on its own in single system LCH. If LCH of the skin is suspected a skin biopsy can confirm. 
 

Lesions  - on the skin may present as reddish papules that progress and ulcerate, or depigmentate and heal. Sometimes mistaken for cradle cap or seborrhic dermatitis.

 

Painful swelling of bones and surrounding tissue;  often in the arms, shoulder (scapula) and leg - a limp is common; hands and feet are rarely involved, although it is possible, see below;


: lesions on the skull

 

Wheezing, coughing, shortness of breath; If the lungs are involved, this can be seen in adults with LCH.

Chornic ear and mastoid infections.
 

Enlarged Lymph nodes.

Excessive thirst and urinating;  if the pituitary gland is affected, causing Diabetes Insipudus. For information on Diabetes Insipidus and related problems please go to pituitary.org.uk

Prolonged vomiting and diarrhoea with blood and mucous, weight loss/prolonged stomach pains; Gastrointestinal tract involvement of LCH is very rare. It can part of a generalised disease, or as a separate primary entity.

Bulging eyes.

A child may have one or many of the above symptoms - they are a guide

 

 

useful links

Cancer Backup -  LCH in children click here

Langerhans Cell Histiocytosis in Children, American Histiocytosis Association of America click here 

 

 

 

Sara Wort has set up a group for her daughter Ebony, who has Langerhans Cell Histiocytosis, on facebook. Please take a look and offer your support.