Loading
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.
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.
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.
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.
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: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, 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
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
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.
The 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.
Query 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.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Dabrafenib: take on an empty stomach. Trametinib: take on an empty stomach; both cause pyrexia.
Severe photosensitivity: sun protection.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
Several lymphoma treatments knock out the part of the immune system that keeps a particular lung infection, Pneumocystis, at bay. A low-dose antibiotic three times a week prevents it, and a separate tablet prevents shingles.
See all on the product pages:CladribineCobimetinibDabrafenib + trametinibVemurafenibVinblastine·Printable cards in the navigator
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.
Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.