Disease · MONDO_0018310
Langerhans cell histiocytosis
6 sourcesdiseases-2026-09-18-0.1.0
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ConductScience Library. Langerhans cell histiocytosis. Diseases, record MONDO_0018310. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0018310
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Disease
Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.
- Other names
LCH, Langerhan's cell histiocytosis, Langerhans cell granulomatosis, Langerhans cell histiocytosis, NOS, Langerhans cell histiocytosis, Not otherwise specified, histiocytosis X, letterer-Siwe disease involving intra-abdominal lymph nodes, letterer-Siwe disease involving intrapelvic lymph nodes, letterer-Siwe disease involving intrathoracic lymph nodes, letterer-Siwe disease involving lymph nodes of axilla and upper limb, letterer-Siwe disease involving lymph nodes of head, face and neck, letterer-Siwe disease involving lymph nodes of inguinal region and lower limb, letterer-Siwe disease involving lymph nodes of multiple sites, letterer-Siwe disease involving spleen, letterer-Siwe disease of intra-abdominal lymph nodes, letterer-Siwe disease of intrapelvic lymph nodes, letterer-Siwe disease of intrathoracic lymph nodes, letterer-Siwe disease of lymph nodes of axilla and upper limb, letterer-Siwe disease of lymph nodes of axilla and/or upper limb, letterer-Siwe disease of lymph nodes of head, face and neck, letterer-Siwe disease of lymph nodes of head, face and/or neck, letterer-Siwe disease of lymph nodes of inguinal region amd/or lower limb, letterer-Siwe disease of lymph nodes of inguinal region and lower limb, letterer-Siwe disease of lymph nodes of inguinal region and/or lower limb, letterer-Siwe disease of lymph nodes of multiple sites, letterer-Siwe disease of spleen
- Open Targets
Drugs35
The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.
| Drug | ChEMBL ID | Type | Open Targets' stage | Stage reported by | Reports | Of them trials | Trials shown here | Source |
|---|---|---|---|---|---|---|---|---|
| CYTARABINE | CHEMBL803 | Small molecule | Phase 3 | ClinicalTrials.gov | 7 | 7 | 7 | Drugs and targets |
| METHOTREXATE | CHEMBL34259 | Small molecule | Phase 3 | ClinicalTrials.gov | 5 | 5 | 5 | Drugs and targets |
| PREDNISONE | CHEMBL635 | Small molecule | Phase 3 | ClinicalTrials.gov | 5 | 5 | 5 | Drugs and targets |
| MERCAPTOPURINE | CHEMBL1200751 | Small molecule | Phase 3 | ClinicalTrials.gov | 4 | 4 | 3 | Drugs and targets |
| VINBLASTINE | CHEMBL159 | Small molecule | Phase 3 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| VINCRISTINE | CHEMBL90555 | Small molecule | Phase 3 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| LEUCOVORIN | CHEMBL1679 | Small molecule | Phase 3 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
| CLADRIBINE | CHEMBL1619 | Small molecule | Phase 2/3 | ClinicalTrials.gov | 2 | 2 | 2 | Drugs and targets |
| HUMAN IMMUNOGLOBULIN G | CHEMBL1201599 | Phase 2/3 | ClinicalTrials.gov | 1 | 1 | 0 | Drugs and targets | |
| INDOMETHACIN | CHEMBL6 | Small molecule | Phase 2/3 | ClinicalTrials.gov | 1 | 1 | 1 | Drugs and targets |
1 to 10 of 35
See all 35 drugsTargets54
The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.
| Target | Protein | Its drugs that act on it | Mechanisms | Highest stage of those drugs | Gene in Genes | Source |
|---|---|---|---|---|---|---|
| BRAF | RAF serine/threonine protein kinase | 3 | RAF serine/threonine protein kinase inhibitor; Serine/threonine-protein kinase B-raf inhibitor | Phase 2 | https://conductscience.org/library/genes/673 | Drugs and targets |
| MAP2K1 | Dual specificity mitogen-activated protein kinase kinase 1 | 3 | Dual specificity mitogen-activated protein kinase kinase 1 inhibitor; Dual specificity mitogen-activated protein kinase kinase; MEK1/2 inhibitor | Phase 2 | https://conductscience.org/library/genes/5604 | Drugs and targets |
| MAP2K2 | Dual specificity mitogen-activated protein kinase kinase 2 | 3 | Dual specificity mitogen-activated protein kinase kinase 2 inhibitor; Dual specificity mitogen-activated protein kinase kinase; MEK1/2 inhibitor | Phase 2 | https://conductscience.org/library/genes/5605 | Drugs and targets |
| NR3C1 | Glucocorticoid receptor | 3 | Glucocorticoid receptor agonist | Phase 3 | https://conductscience.org/library/genes/2908 | Drugs and targets |
| POLA1 | DNA polymerase (alpha/delta/epsilon) | 3 | DNA polymerase (alpha/delta/epsilon) inhibitor | Phase 3 | https://conductscience.org/library/genes/5422 | Drugs and targets |
| POLA2 | DNA polymerase (alpha/delta/epsilon) | 3 | DNA polymerase (alpha/delta/epsilon) inhibitor | Phase 3 | https://conductscience.org/library/genes/23649 | Drugs and targets |
| POLD1 | DNA polymerase (alpha/delta/epsilon) | 3 | DNA polymerase (alpha/delta/epsilon) inhibitor | Phase 3 | https://conductscience.org/library/genes/5424 | Drugs and targets |
| POLD2 | DNA polymerase (alpha/delta/epsilon) | 3 | DNA polymerase (alpha/delta/epsilon) inhibitor | Phase 3 | https://conductscience.org/library/genes/5425 | Drugs and targets |
| POLD3 | DNA polymerase (alpha/delta/epsilon) | 3 | DNA polymerase (alpha/delta/epsilon) inhibitor | Phase 3 | https://conductscience.org/library/genes/10714 | Drugs and targets |
| POLD4 | DNA polymerase (alpha/delta/epsilon) | 3 | DNA polymerase (alpha/delta/epsilon) inhibitor | Phase 3 | https://conductscience.org/library/genes/57804 | Drugs and targets |
1 to 10 of 54
See all 54 targetsTrials34
Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.
| Trial | Title | Phase | Participants | Results first posted | Conditions | Drugs | Linked by | Source |
|---|---|---|---|---|---|---|---|---|
| NCT01524978 | A Study of Vemurafenib in Participants With BRAF V600 Mutation-Positive Cancers | Phase 2 | 208 | 2017-11-20 | Multiple Myeloma, Neoplasms | CETUXIMAB, VEMURAFENIB | Its record names the disease | ClinicalTrials.gov |
| NCT02124772 | Study to Investigate Safety, Pharmacokinetic (PK), Pharmacodynamic (PD) and Clinical Activity of Trametinib in Subjects With Cancer or Plexiform Neurofibromas and Trametinib in Combination With Dabrafenib in Subjects With Cancers Harboring V600 Mutations | Phase 1/2 | 139 | 2021-07-14 | Cancer | DABRAFENIB, TRAMETINIB | Its record names the disease | ClinicalTrials.gov |
| NCT01677741 | A Study to Determine Safety, Tolerability and Pharmacokinetics of Oral Dabrafenib In Children and Adolescent Subjects | Phase 1/2 | 85 | 2021-11-24 | Neoplasms, Brain | DABRAFENIB | Its record names the disease | ClinicalTrials.gov |
| NCT02425904 | Study of Clofarabine in Patients With Recurrent or Refractory Langerhans Cell Histiocytosis and LCH-related Disorders | Phase 2 | 25 | 2022-02-16 | Langerhans Cell Histiocytosis | CLOFARABINE | Its record names the disease | ClinicalTrials.gov |
| NCT02523040 | Lenalidomide for Adult Histiocyte Disorders | Phase 2 | 12 | 2025-04-25 | Langerhans Cell Histiocytosis (LCH), Histiocytoses Erdheim-chester Disease, Histiocytic Sarcoma (HS) | LENALIDOMIDE | Its record names the disease | ClinicalTrials.gov |
| NCT00588536 | Study of Sequential Administration of Oral 6-Thioguanine After Methotrexate in Patients With LCH | Phase 2 | 5 | 2015-05-04 | Langerhans Cell Histiocytosis | METHOTREXATE, THIOGUANINE | Its record names the disease | ClinicalTrials.gov |
| NCT00618540 | Reduced Intensity Hematopoietic Cell Transplantation for Patients With Resistant Langerhans Cell Histiocytosis | Phase 2 | 1 | 2015-11-03 | Histiocytosis, Langerhans-cell | ALEMTUZUMAB, FLUDARABINE PHOSPHATE, MELPHALAN | Its record names the disease | ClinicalTrials.gov |
| NCT02205762 | LCH-IV, International Collaborative Treatment Protocol for Children and Adolescents With Langerhans Cell Histiocytosis | Phase 2/3 | 1,400 | Langerhans Cell Histiocytosis | CLADRIBINE, CYTARABINE, INDOMETHACIN, MERCAPTOPURINE, METHOTREXATE, PREDNISONE, VINBLASTINE | Its record names the disease | ClinicalTrials.gov | |
| NCT00276757 | Combination Chemotherapy in Treating Young Patients With Langerhans Cell Histiocytosis | Not applicable | 376 | Childhood Langerhans Cell Histiocytosis | LEUCOVORIN CALCIUM, METHOTREXATE, PREDNISOLONE, VINBLASTINE SULFATE | Its record names the disease | ClinicalTrials.gov | |
| NCT04773366 | A Prospective Study for the Treatment of Children With Newly Diagnosed LCH Using a Cytarabine Contained Protocol | Phase 3 | 200 | Langerhans Cell Histiocytosis | CYTARABINE, MERCAPTOPURINE, PREDNISONE, VINCRISTINE | Its record names the disease | ClinicalTrials.gov |
1 to 10 of 34
See all 34 trialsComparisons in its trials4
Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.
| Comparison | Group 1, with the outcome | Group 2, with the outcome | Participants | Trial | Source |
|---|---|---|---|---|---|
| Cohort 3b: Colorectal Cancer - Vemurafenib + Cetuximab vs Pooled Arm - Vemurafenib: serious adverse events | 11 of 27 | 91 of 181 | 208 | NCT01524978 | Trial comparisons |
| Recurrent or Refractory Langerhans Cell Histiocytosis (LCH) + Clofarabine vs LCH-related Disorders + Clofarabine: Number of Participants With at Least One Grade 3 or Higher Treatment-Related Toxicity | 15 of 20 | 4 of 4 | 24 | NCT02425904 | Trial comparisons |
| Recurrent or Refractory Langerhans Cell Histiocytosis (LCH) + Clofarabine vs LCH-related Disorders + Clofarabine: deaths from any cause | 0 of 20 | 3 of 4 | 24 | NCT02425904 | Trial comparisons |
| Recurrent or Refractory Langerhans Cell Histiocytosis (LCH) + Clofarabine vs LCH-related Disorders + Clofarabine: serious adverse events | 15 of 20 | 4 of 4 | 24 | NCT02425904 | Trial comparisons |
Papers from its trials38
The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.
Data dates
The date of each source this release read.
Sources 6
- ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
- ChEMBL, through Open TargetsCC BY-SA 3.0
- ClinicalTrials.govNo license stated
- NLM's MeSHNLM's terms
- Open TargetsCC0 1.0
- PubMedNLM's terms
Each source's terms
- ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
- ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
- ClinicalTrials.govnone stated
- NLM's MeSHNLM's
- Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
- PubMedNLM's terms
ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.
For software
Read this disease from software.
The record and each of its lists are JSON, twenty rows a page. No key.
GET https://conductscience.org/api/diseases/v1/MONDO_0018310
GET https://conductscience.org/api/diseases/v1/MONDO_0018310/drugs?page=2