Single-system Langerhans cell histiocytosis (bone, skin or one other organ)
Single-system Langerhans cell histiocytosis is the milder form of this rare histiocytosis, in which the abnormal immune cells affect only one organ, usually a bone or the skin, in a child or young adult. Single bone lesions often heal after biopsy or curettage, skin disease may fade by itself, and gentle vinblastine and prednisone is kept for multiple bone lesions or lesions near the brain.
Overview
Langerhans cell histiocytosis is a clonal myeloid neoplasm of CD1a- and langerin-positive dendritic-like cells, driven in most cases by BRAF V600E or another MAPK pathway mutation. Its behaviour depends on how many organs are involved. Single-system disease, most often a lytic bone lesion of the skull, femur, ribs or vertebrae in a child of school age, or a skin eruption in an infant, carries almost no mortality; the classic eponyms eosinophilic granuloma (bone), Hand-Schüller-Christian and Letterer-Siwe disease have given way to a classification by organ count and risk-organ involvement. Pulmonary Langerhans cell histiocytosis in adults who smoke is a distinct single-system form that often improves with smoking cessation. The Histiocyte Society staging separates unifocal bone disease, multifocal bone disease, special-site lesions (skull base, orbit, mastoid and vertebrae with soft tissue extension, which carry a risk of later pituitary or neurodegenerative involvement) and single-system disease of skin, lymph node or lung.
Treatment is proportionate. A single bone lesion is treated by biopsy with curettage, sometimes with an intralesional steroid injection, and many heal spontaneously; indomethacin or bisphosphonates help painful bone disease; skin-only disease in infants is observed or treated topically and often resolves, though a proportion of infants later develop multisystem disease and must be followed. Multifocal bone disease and special-site lesions are treated with the same vinblastine and prednisone regimen used for multisystem disease, given for twelve months after LCH-III showed longer treatment reduced reactivation, in order to prevent recurrence and the late central nervous system complications. Reactivation is common but rarely dangerous. Adults with single-system disease may receive cytarabine or cladribine instead because vinblastine is more toxic in adults, and BRAF or MEK inhibitors are reserved for refractory disease. The main long-term concerns are diabetes insipidus and neurodegenerative disease after skull-base lesions, and orthopaedic sequelae after vertebral collapse, so follow-up continues for years.
State of the art
- Most single-system disease is cured with minimal treatment and near-zero mortality.
- Special-site lesions are treated systemically to prevent the late neurological complications.
- BRAF testing links the mildest and the most severe forms of the disease as one neoplasm.
Red cards
From the labels and guidelines behind the standard of care. Your team's thresholds win.- Emergency services nowFainting or palpitations
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
- Emergency services nowBleeding or bruising
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
- Check before combiningFood and drink: Dabrafenib + trametinib
Dabrafenib: take on an empty stomach. Trametinib: take on an empty stomach; both cause pyrexia.
- Check before combiningFood and drink: Vemurafenib
Severe photosensitivity: sun protection.
- Check before combiningHeart rhythm (QT): Vemurafenib
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
See all on the product pages:CladribineCobimetinibDabrafenib + trametinibVemurafenibVinblastine·Printable cards in the navigator
Anatomy and lymph node drainage
- Bone marrow (leukaemia, MDS, MPN, myeloma)
- Lymph node germinal centre (lymphomas)
- Spleen
- Blood (leukaemic phase)
- Lytic bone lesions (myeloma)
- Skin and extranodal sites
- Nodes: cervical
- Nodes: axillary
- Nodes: mediastinal
- Nodes: para-aortic and mesenteric
- Nodes: inguinal
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
- Bone marrow (leukaemia, MDS, MPN, myeloma)Unifocal bone Langerhans cell histiocytosis (eosinophilic granuloma; curettage or observation) · Multifocal bone Langerhans cell histiocytosis (vinblastine and prednisone) · Special-site Langerhans cell histiocytosis (skull base, orbit, mastoid, vertebra; treated to prevent CNS complications) · Skin-only Langerhans cell histiocytosis in infants (observation, may progress) · Single-system lymph node or thymic Langerhans cell histiocytosis · Pulmonary Langerhans cell histiocytosis in adult smokers (smoking cessation)
- Lymph node germinal centre (lymphomas)
- Spleen
- Blood (leukaemic phase)
- Lytic bone lesions (myeloma)
- Skin and extranodal sitesSpecial-site Langerhans cell histiocytosis (skull base, orbit, mastoid, vertebra; treated to prevent CNS complications) · Skin-only Langerhans cell histiocytosis in infants (observation, may progress)
- cervical
- axillary
- mediastinal
- para-aortic and mesenteric
- inguinal
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
About two thirds of childhood Langerhans cell histiocytosis is confined to one organ system, most often bone; the outlook is excellent and many lesions heal with minimal or no treatment.
- MRIStandard of care
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Biopsy with immunohistochemistry and BRAF testing; skeletal survey or whole-body imaging, blood count, liver tests and abdominal ultrasound to exclude multisystem disease.
Biopsy with curettage, with or without intralesional methylprednisolone; observation of healing; indomethacin for pain.
Vinblastine and prednisone for twelve months (LCH-III schedule) to reduce reactivation and central nervous system risk.
Observation or topical corticosteroids; systemic therapy only for extensive symptomatic disease; regular review for progression to multisystem disease.
Cytarabine or cladribine; smoking cessation for pulmonary disease; BRAF or MEK inhibitors (vemurafenib, dabrafenib-trametinib, cobimetinib) for refractory disease.
Subtypes & biomarkers
top- Unifocal bone Langerhans cell histiocytosis (eosinophilic granuloma; curettage or observation)
- Multifocal bone Langerhans cell histiocytosis (vinblastine and prednisone)
- Special-site Langerhans cell histiocytosis (skull base, orbit, mastoid, vertebra; treated to prevent CNS complications)
- Skin-only Langerhans cell histiocytosis in infants (observation, may progress)
- Single-system lymph node or thymic Langerhans cell histiocytosis
- Pulmonary Langerhans cell histiocytosis in adult smokers (smoking cessation)
- CD1a and langerin (CD207) positive histiocytes on biopsy
- BRAF V600E in tissue (present in over half) and cell-free DNA
- MAP2K1 and other MAPK alterations
- Skeletal survey or whole-body MRI or PET for occult lesions
- Pituitary MRI and water balance for special-site disease
- Chest CT and pulmonary function in adult pulmonary disease
How often this target appears
- 1940Eosinophilic granuloma of bone described by Lichtenstein and Jaffe
- 1953Lichtenstein unifies eosinophilic granuloma, Hand-Schüller-Christian and Letterer-Siwe disease as histiocytosis X
- 1987Histiocyte Society founded; disease renamed Langerhans cell histiocytosis
- 2010BRAF V600E found in over half of lesions
- 2013LCH-III: twelve months of vinblastine and prednisone reduces reactivation
Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 7 changes by month →- 2026-09-18This recordSingle-system Langerhans cell histiocytosis (bone, skin or one other organ)Facts on this page last checked
When this page itself was last checked or edited.
- 2013Trial resultLCH-IIILCH-III reported
12 vs 6 months of vinblastine-prednisone: 5-year reactivation 37% vs 54% in risk-organ-negative disease; methotrexate added no benefit.
- 2013MilestoneLCH-IIILCH-III: twelve months of vinblastine and prednisone reduces reactivation
A milestone in how this cancer is treated.
- 2010MilestoneBRAFBRAF V600E found in over half of lesions
A milestone in how this cancer is treated.
- 1987MilestoneSingle-system Langerhans cell histiocytosis (bone, skin or one other organ)Histiocyte Society founded; disease renamed Langerhans cell histiocytosis
A milestone in how this cancer is treated.
- 1953MilestoneSingle-system Langerhans cell histiocytosis (bone, skin or one other organ)Lichtenstein unifies eosinophilic granuloma, Hand-Schüller-Christian and Letterer-Siwe disease as histiocytosis X
A milestone in how this cancer is treated.
What is in development for Single-system Langerhans cell histiocytosis (bone, skin or one other organ), drawn from the whole corpus: 1 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Trials reported · 1
- LCH-III · phase 3 · 2013 · positive
Open problems and what is being done
Which infants with skin-only disease will progress cannot be predicted.
Whether treating special-site lesions truly prevents neurodegeneration is inferred rather than proven.
Adults have no trial-based standard.
Reactivation rates remain high even after twelve months of therapy.
Trials
topTrials recruiting now
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Landmark trials
Expert centres
topExpert centres
Boston · hospital | United States | 0 | 3,582 | 54,857 | #16 | ||
Pitman · consortium | United States | none recorded | 1 | not matched | - | none recorded | - |
Barcelona · hospital | Spain | none recorded | 0 | 1,265 | 15,631 | - | |
Lyon · cancer center | France | none recorded | 0 | 1,071 | 14,301 | - | |
Changsha · cancer center | China | none recorded | 0 | 930 | 14,477 | - | |
Taipei · hospital | Taiwan | none recorded | 0 | 746 | 8,966 | - | |
Guangzhou · hospital | China | none recorded | 0 | 704 | 7,832 | - | |
Shanghai · hospital | China | none recorded | 0 | 539 | 6,491 | - | |
Ghent · hospital | Belgium | none recorded | 0 | 495 | 5,334 | - | |
Aurora, CO · cancer center | United States | 0 | 483 | 13,480 | - | ||
Shanghai · hospital | China | none recorded | 0 | 464 | 6,795 | - | |
Southampton · hospital | United Kingdom | none recorded | 0 | 434 | 3,990 | - | |
Leeds · hospital | United Kingdom | none recorded | 0 | 363 | 6,609 | - | |
Candiolo · cancer center | Italy | none recorded | 0 | 324 | 4,466 | - | |
Sendai · hospital | Japan | none recorded | 0 | 161 | 936 | - |
These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Single-system Langerhans cell histiocytosis but is thinly recorded here will look small.
Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.
Questions to ask
topQuestions to ask your oncologist about Single-system Langerhans cell histiocytosis
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example CD1a and langerinpositive histiocytes on biopsy, BRAF V600E in tissueand cell-free DNA, MAP2K1 and other MAPK alterations, Skeletal survey or whole-body MRI or PET for occult lesions, Pituitary MRI and water balance for special-site disease), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Unifocal bone Langerhans cell histiocytosis, Multifocal bone Langerhans cell histiocytosis, Special-site Langerhans cell histiocytosis.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Diagnosis and staging
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Why: Guideline options include: Biopsy with immunohistochemistry and BRAF testing; skeletal survey or whole-body imaging, blood count, liver tests and abdominal ultrasound to exclude multisystem disease.
Unifocal bone disease
- For my situation (unifocal bone disease), which of the standard options do you recommend and why?Why: Guideline options include: Biopsy with curettage, with or without intralesional methylprednisolone; observation of healing; indomethacin for pain.
Multifocal bone or special-site disease
- For my situation (multifocal bone or special-site disease), which of the standard options do you recommend and why?Why: Guideline options include: Vinblastine and prednisone for twelve months (LCH-III schedule) to reduce reactivation and central nervous system risk.
- Am I a candidate for Vinblastine, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of LCH-III apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
Skin-only disease
- For my situation (skin-only disease), which of the standard options do you recommend and why?Why: Guideline options include: Observation or topical corticosteroids; systemic therapy only for extensive symptomatic disease; regular review for progression to multisystem disease.
Adult single-system or refractory disease
- For my situation (adult single-system or refractory disease), which of the standard options do you recommend and why?Why: Guideline options include: Cytarabine or cladribine; smoking cessation for pulmonary disease; BRAF or MEK inhibitors (vemurafenib, dabrafenib-trametinib, cobimetinib) for refractory disease.
- Am I a candidate for Cladribine, Vemurafenib, Dabrafenib + trametinib or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Dabrafenib + trametinib, Vemurafenib, Cobimetinib?Why: Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Which infants with skin-only disease will progress cannot be predicted”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Whether treating special-site lesions truly prevents neurodegeneration is inferred rather than proven”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Newly diagnosed? Read the first 60 days with Single-system Langerhans cell histiocytosis, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
7targets
1drugs
5companies
4pathways
1terms
2trials
1key papers
3The way OnCo groups the histiocytoses, and the recognition that LCH and ECD are cancers rather than inflammatory conditions, come from this classification.
Children with multisystem LCH receive a year of vinblastine and prednisone, and those in whom the disease does not respond quickly are switched early to salvage therapy.
LCH is a MAPK-pathway-driven neoplasm; BRAF testing is now routine and BRAF and MEK inhibitors are used for refractory disease.
Latest papers
topQuery for this cancer: (TITLE:"Single-system Langerhans cell histiocytosis" OR ABSTRACT:"Single-system Langerhans cell histiocytosis" OR TITLE:"bone, skin or one other organ" OR ABSTRACT:"bone, skin or one other organ" OR TITLE:"Single-system LCH" OR ABSTRACT:"Single-system LCH" OR TITLE:"Eosinophilic granuloma" OR ABSTRACT:"Eosinophilic granuloma" OR TITLE:"Unifocal bone LCH" OR ABSTRACT:"Unifocal bone LCH" OR TITLE:"Skin-only LCH" OR ABSTRACT:"Skin-only LCH") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Single-system Langerhans cell histiocytosis (bone, skin or one other organ), not a curated reading list.
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