Langerhans' cell histiocytosis (LCH) in children

Macmillan and Cancerbackup have merged.Together we provide free, high quality information for all.Information developed jointly with theChildren's Cancer and Leukaemia GroupLangerhans' cell histiocytosis (LCH) in childrenThis fact sheet is about a conditioncalled Langerhans' cell histiocytosis (LCH)in children. We hope it answers yourquestions. If you have any further questions,you can ask a nurse or doctor involved inyour child's treatment.If you'd like to discuss this informationwith our cancer support specialists,call the Macmillan Support Line free on0808 808 00 00, Monday–Friday,9am–8pm. If you're hard of hearing youcan use textphone 0808 808 0121,or Text Relay. For non-English speakers,interpreters are available. Alternatively,visit macmillan.org.ukIncludes the following information• Langerhans' cell histiocytosis (LCH)• Causes of LCH• Signs and symptoms• How LCH is diagnosed• Treatment• Side effects of treatment• Late side effects• Clinical trials• Follow-up• Your feelings• Useful organisations• Related Macmillan informationLangerhans' cell histiocytosis (LCH)LCH is not strictly a cancer, but rather acancer-like condition that may be treatedwith chemotherapy. In its more seriousforms, LCH can behave like a cancer andis therefore usually treated by children'scancer specialists (paediatric oncologists).Langerhans' refers to Dr Paul Langerhans,who first described the cells in the skinfrom which LCH develops.Histiocytes are cells which are part of theimmune system and are found in manyparts of the body. There are two types ofhistiocytes: macrophage monocyte cells,which destroy harmful proteins, viruses andPage 1 of 5 Macmillan fact sheet 2012: Langerhans' cell histiocytosis (LCH) in children

acteria in the body; and dendritic cells,which stimulate the immune system.Langerhans' cells are dendritic cells andare normally only found in the skin andmajor airways. In LCH, the Langerhans'cells are abnormal and spread via thebloodstream into many parts of the body,including the bone marrow, skin, lungs,liver, lymph glands, spleen and pituitarygland. When Langerhans' cells are presentin these tissues, they may cause damage.LCH is divided into two groups:• single-system LCH, when the diseaseaffects only one part of the body,for example, the skin or the bone• multi-system LCH, when it affects morethan one part of the body.About 50 children in the UK developLangerhans' cell histiocytosis (LCH) eachyear. It can affect children of any age,and is more common in boys than in girls.Causes of LCHThe cause of LCH is unknown. It cannotbe caught from other people and is notpassed on in families.Signs and symptomsThe symptoms of LCH will depend onwhich part of the body is affected andwhether the disease is affecting more thanone part of the body.If LCH affects the bone, this can causepain in the bone and/or swelling/lumps onthe skull. If the skin is affected, a skin rash,such as cradle cap and nappy rash, mayoccur. A discharge from the ear or hearingproblems can occur if the ear is affected.If LCH affects the lungs or chest, the childmay have breathing difficulties. LCH withinthe abdomen may cause tummy problems,such as diarrhoea, and liver problems,including jaundice. The lymph glands maybe enlarged. Children may be irritable andhave a poor appetite.In 2–3 out of every 10 children withmulti-system disease, the pituitary glandat the base of the brain is affected, causinghormonal problems. This can lead to thechild passing larger amounts of urine andbeing very thirsty. This is called diabetesinsipidus, which is different from sugardiabetes and can be well controlled withspecific medicines. Occasionally, otherpituitary hormones may be affectedcausing poor growth or delayed puberty,which again can be treated.How LCH is diagnosedA variety of tests and investigations maybe needed to diagnose LCH. X-rays willoften be taken of the bones, the skull,and the lungs. Blood tests will also betaken. These tests help the doctors todecide if the disease is a single-systemor multi‐system type.Tests are likely to include the removal of asample of cells (biopsy), and this is usuallydone in an operation, under a generalanaesthetic. The cells are examinedunder a microscope. An MRI (magenticresonance imaging) scan of the brain mayalso be carried out.Any tests and investigations that your childneeds will be explained to you. The bookletA parent’s guide to children’s cancers, givesdetails of what the tests and scans involve.TreatmentSingle-system LCH may disappear onits own without any treatment. Thismay occur following a biopsy. In somechildren, treatment such as surgery andcorticosteroids (such as prednisolone) maybe used.Multi-system disease is usually treatedwith chemotherapy and corticosteroids.The length of treatment varies from childto child.Chemotherapy is the use of anti-cancer(cytotoxic) drugs to destroy cancercells, and corticosteroids are hormonalPage 2 of 5 Macmillan fact sheet 2012: Langerhans' cell histiocytosis (LCH) in children

substances which are naturally producedin the body. Both chemotherapy andcorticosteroids can help to destroy theLCH cells.Side effects of treatmentChemotherapy and corticosteroids usedto treat LCH may cause side effects, andyour child's doctor will discuss these withyou before treatment starts. Any possibleside effects will depend upon the particulartreatment being used and the part of thebody that is being treated.Side effects of chemotherapy can include:feeling sick (nausea) and vomiting, hairloss, an increased risk of bruising andbleeding, and tiredness. Corticosteroidsmay cause a rounding of the face(sometimes called a moon face), moodchanges, and an increased appetite, whichcan lead to weight gain. All of these sideeffects are reversible when treatment stops.Late side effectsSome children may develop after-effectsof the disease. These effects can happenwithin months or years of the diagnosisand are more likely to be due to thedisease itself than to the treatment given.However, a few children may develop lateeffects from the drugs or the treatmentthey receive.Possible late side effects include reductionin bone growth, delayed puberty, hearingproblems, and lung problems.Clinical trialsMany children have their treatment as partof a clinical research trial. Trials aim toimprove our understanding of the best wayto treat an illness, usually by comparing thestandard treatment with a new or modifiedversion. Research has found that peoplereceiving treatment within clinical trials tendto do as well as, or better than, similarpatients treated outside clinical trials.This does not mean that trial treatmentsare always better; hospitals that carry outtrials do however have access to goodequipment and follow precise guidelineswhen giving treatment.The treatment trials for LCH are set upand organised by The Histiocyte Society,a group of international specialists inhistiocytosis. If appropriate, your child'smedical team will talk to you about takingpart in a clinical trial and will answerany questions you may have. Writteninformation is often provided to helpexplain things.Taking part in a research trial is completelyvoluntary, and you'll be given time todecide if it's right for your child.Follow-upBetween eight and nine of every 10children who develop LCH will recoverfrom it. Sometimes the disease can comeback, and so children will have regularcheck-ups in the outpatients clinic.Follow-up is also important to look outfor late side effects. If you have specificconcerns about your child’s condition andtreatment, it's best to discuss them withyour child’s specialist medical team whoknow the situation in detail.Your feelingsAs a parent, the fact that your child has aserious illness is one of the worst situationsthat you can be faced with. You may havemany different emotions, such as fear, guilt,sadness, anger and uncertainty. These areall normal reactions and are part of theprocess that many parents go through atsuch a difficult time.It's not possible to address in this factsheet all of the feelings you may have.However, the Macmillan/CCLG bookletA parent’s guide to children’s cancers talksabout the emotional impact of caringfor an unwell child and suggests sourcesof help and support.Page 3 of 5 Macmillan fact sheet 2012: Langerhans' cell histiocytosis (LCH) in children

Your child may have a range of powerfulemotions throughout their experienceof illness. The parent's guide discussesthis further and talks about how you cansupport your child.Our booklet Peppermint Ward is astorybook for younger children with cancer.It looks at the issues they and their familymay face, and helps them explore theirfeelings.Useful organisationsChildren's Cancer and LeukaemiaGroup (CCLG)University of Leicester, 3 rd Floor, Heartsof Oak House, 9 Princess Road West,Leicester LE1 6THTel 0116 249 4460 (main office)Email info@cclg.org.ukwww.cclg.org.ukCoordinates research and care for childrenand their parents. There are 21 CCLGspecialist centres for the treatment ofchildhood cancer and leukaemia, coveringall areas of the UK and Ireland (there's amap of the centres on the website). Hasinformation about the CCLG, childhoodcancer and leukaemia.CLIC SargentGriffin House, 161 Hammersmith Road,London W6 8SGTel 0300 330 0803Email info@clicsargent.org.ukwww.clicsargent.org.ukOffers practical support to children andyoung people with cancer or leukaemia,and to their families.The Histiocytosis Associationwww.histio.orgAn international partnership of patients,families, physicians and friends, whichaims to promote scientific research andeducation related to histiocytosis. It aims toprovide solutions to some of the problemsthat are specific to patients suffering fromthis disease, and to offer support to patientsand their families.Histiocytosis Research Trust18 Western Road, Wylde Green, SuttonColdfield B73 5SPTel 0121 355 5137Email info@hrtrust.orgwww.hrtrust.orgFormed to promote and fund scientificresearch into histiocytoses. The trust hasalso created a ‘Circle of Friends’ for mutualsupport through information, newslettersand roadshows led by medical experts.In touch with many families around theUK and overseas.Related Macmillan information• A parent's guide to children's cancers• Katie's Garden (a storybook for primaryschool-age children about a girl'sexperience of cancer)• Peppermint Ward – a story aboutchildren with cancer• Understanding cancer research trials(clinical trials)• Understanding chemotherapy• Understanding radiotherapyFor copies of this related information callfree on 0808 808 00 00, or see it onlineat macmillan.org.ukOur website click4tic.org.uk has cancerinformation written specifically for youngpeople. Get advice and support, andconnect with other young people affectedby cancer.This fact sheet has been written, revised and editedby Macmillan Cancer Support’s Cancer InformationDevelopment team. It has been approved by ourmedical editor, Dr Terry Priestman, ConsultantClinical Oncologist.With thanks to: Dr Kevin Windebank, SeniorLecturer in child health & Consultant paediatric andadolescent oncologist; and the people affected bycancer who reviewed this edition.Page 4 of 5 Macmillan fact sheet 2012: Langerhans' cell histiocytosis (LCH) in children

This fact sheet has been compiled using informationfrom a number of reliable sources, including:• Voute PA, et al. Cancer in Children: ClinicalManagement. 5 th edition. 2005. OxfordUniversity Press.• Pinkerton, et al. Evidence-based paediatriconcology. 2 nd edition. 2007. BlackwellPublishing.• McLain KL. Langerhans cell histiocytosis,Juvenile xanthogranuloma, and Erdheim-Chester disease. www.uptodate.com(accessed October 2010).This fact sheet was revised in 2011. The next editionwill be available in 2013.We make every effort to ensure that theinformation we provide is accurate but it shouldnot be relied upon to reflect the current state ofmedical research, which is constantly changing.If you are concerned about your health, youshould consult your doctor. Macmillan cannotaccept liability for any loss or damage resultingfrom any inaccuracy in this information orthird-party information such as information onwebsites to which we link.© Macmillan Cancer Support 2012. Registeredcharity in England and Wales (261017),Scotland (SC039907) and the Isle of Man(604). Registered office 89 Albert Embankment,London, SE1 7UQ.MAC11870_12Page 5 of 5 Macmillan fact sheet 2012: Langerhans' cell histiocytosis (LCH) in children