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Disease · MONDO_0002083

Richter transformation

6 sourcesdiseases-2026-09-18-0.1.0

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ConductScience Library. Richter transformation. Diseases, record MONDO_0002083. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0002083

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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.

Name
Richter transformation
Open Targets
Open Targets ID
MONDO_0002083
Open Targets
Other names
Richter syndrome, Richter's syndrome, Richter's transformation, chronic lymphocytic leukemia with Richter transformation, chronic lymphocytic leukemia/small lymphocytic lymphoma with Richter transformation
Open Targets
Disease Ontology
DOID:1703
Disease Ontology
NCI Thesaurus
C35424
NCI Thesaurus
Highest stage of its drugs
Phase 3, for 6 of its 53 drugs, from ClinicalTrials.gov
Open Targets

Drugs53

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.

DrugChEMBL IDTypeOpen Targets' stageStage reported byReportsOf them trialsTrials shown hereSource
RITUXIMABCHEMBL1201576AntibodyPhase 3ClinicalTrials.gov111111Drugs and targets
CYCLOPHOSPHAMIDECHEMBL1200796Small moleculePhase 3ClinicalTrials.gov101010Drugs and targets
PREDNISONECHEMBL635Small moleculePhase 3ClinicalTrials.gov777Drugs and targets
VINCRISTINECHEMBL90555Small moleculePhase 3ClinicalTrials.gov555Drugs and targets
PIRTOBRUTINIBCHEMBL4650485Small moleculePhase 3ClinicalTrials.gov444Drugs and targets
DOXORUBICIN HYDROCHLORIDECHEMBL359744Small moleculePhase 3ClinicalTrials.gov333Drugs and targets
VENETOCLAXCHEMBL3137309Small moleculePhase 2ClinicalTrials.gov101010Drugs and targets
IBRUTINIBCHEMBL1873475Small moleculePhase 2ClinicalTrials.gov887Drugs and targets
NIVOLUMABCHEMBL2108738AntibodyPhase 2ClinicalTrials.gov888Drugs and targets
OBINUTUZUMABCHEMBL1743048AntibodyPhase 2ClinicalTrials.gov888Drugs and targets

1 to 10 of 53

See all 53 drugs

Targets53

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.

TargetProteinIts drugs that act on itMechanismsHighest stage of those drugsGene in GenesSource
MS4A1B-lymphocyte antigen CD208B-lymphocyte antigen CD20 binding agentPhase 3https://conductscience.org/library/genes/931Drugs and targets
BTKTyrosine-protein kinase BTK5Tyrosine-protein kinase BTK inhibitorPhase 3https://conductscience.org/library/genes/695Drugs and targets
CD3DT cell surface glycoprotein CD35T cell surface glycoprotein CD3 binding agent; T cell surface glycoprotein CD3 cross-linking agentPhase 2https://conductscience.org/library/genes/915Drugs and targets
CD3ET cell surface glycoprotein CD35T cell surface glycoprotein CD3 binding agent; T cell surface glycoprotein CD3 cross-linking agentPhase 2https://conductscience.org/library/genes/916Drugs and targets
CD3GT cell surface glycoprotein CD35T cell surface glycoprotein CD3 binding agent; T cell surface glycoprotein CD3 cross-linking agentPhase 2https://conductscience.org/library/genes/917Drugs and targets
CD19B-lymphocyte antigen CD194B-lymphocyte antigen CD19 binding agent; B-lymphocyte antigen CD19 cross-linking agentPhase 2https://conductscience.org/library/genes/930Drugs and targets
CD274Programmed cell death 1 ligand 13Programmed cell death 1 ligand 1 inhibitorPhase 2https://conductscience.org/library/genes/29126Drugs and targets
NR3C1Glucocorticoid receptor3Glucocorticoid receptor agonistPhase 3https://conductscience.org/library/genes/2908Drugs and targets
PDCD1Programmed cell death protein 13Programmed cell death protein 1 inhibitorPhase 2https://conductscience.org/library/genes/5133Drugs and targets
TOP2ADNA topoisomerase 2-alpha3DNA topoisomerase II alpha inhibitor; DNA topoisomerase II inhibitorPhase 3https://conductscience.org/library/genes/7153Drugs and targets

1 to 10 of 53

See all 53 targets

Trials52

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.

TrialTitlePhaseParticipantsResults first postedConditionsDrugsLinked bySource
NCT01607892Safety Study of the Selective Inhibitor of Nuclear Export (SINE) KPT-330 in Patients With Advanced Hematological CancerPhase 12862021-04-01Hematological MalignanciesSELINEXORIts record names the diseaseClinicalTrials.gov
NCT02329847A Study to Evaluate Safety, Pharmacokinetics, Pharmacodynamics and Preliminary Efficacy of the Combination of Ibrutinib With Nivolumab in Participants With Hematologic MalignanciesPhase 1/21442023-06-15Hematologic NeoplasmsIBRUTINIB, NIVOLUMABIts record names the diseaseClinicalTrials.gov
NCT00472849Oxaliplatin, Fludarabine, Cytarabine, and Rituximab in Patients With Richter's Transformation and LeukemiasPhase 1/2922013-11-26Richter's Transformation, LeukemiaCYTARABINE, FLUDARABINE, OXALIPLATIN, RITUXIMABIts record names the diseaseClinicalTrials.gov
NCT02576990Study of Pembrolizumab (MK-3475) in Participants With Relapsed or Refractory Primary Mediastinal Large B-cell Lymphoma or Relapsed or Refractory Richter Syndrome (MK-3475-170/KEYNOTE-170)Phase 2802020-06-24Mediastinal Large B-cell Lymphoma, Richter SyndromePEMBROLIZUMABIts record names the diseaseClinicalTrials.gov
NCT00452374Oxaliplatin, Fludarabine, Cytarabine and Rituximab in Richter's Syndrome, Refractory CLL and PLLPhase 1/2482011-08-29LeukemiaCYTARABINE, FLUDARABINE, OXALIPLATIN, RITUXIMABIts record names the diseaseClinicalTrials.gov
NCT02420912Nivolumab and Ibrutinib in Treating Patients With Relapsed, Refractory, or High-Risk Untreated Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Richter TransformationPhase 2372024-03-20Loss of Chromosome 17p, Recurrent Chronic Lymphocytic Leukemia, Recurrent Small Lymphocytic Lymphoma, Refractory Chronic Lymphocytic Leukemia, Refractory Small Lymphocytic Lymphoma, Richter SyndromeIBRUTINIB, NIVOLUMABIts record names the diseaseClinicalTrials.gov
NCT02138786Selinexor in Initial or Refractory and/or Relapsed Richter's TransformationPhase 2272020-02-11Richter's TransformationSELINEXORIts record names the diseaseClinicalTrials.gov
NCT05537766Study of Brexucabtagene Autoleucel in Adults With Rare B-cell MalignanciesPhase 2192026-03-10Relapsed/Refractory Waldenstrom Macroglobulinemia, Relapsed/Refractory Richter Transformation, Relapsed/Refractory Burkitt Lymphoma, Relapsed/Refractory Hairy Cell LeukemiaBREXUCABTAGENE AUTOLEUCEL, CYCLOPHOSPHAMIDE, FLUDARABINEIts record names the diseaseClinicalTrials.gov
NCT004134785-Azacytidine (Azacytidine; Vidaza) in Chronic Lymphocytic LeukemiaPhase 292015-06-30Chronic Lymphocytic Leukemia, LeukemiaAZACITIDINEIts record names the diseaseClinicalTrials.gov
NCT03121534Ibrutinib, Nivolumab and Blinatumomab in Richter TransformationPhase 292023-02-17Hematopoietic/Lymphoid Cancer, Richter's TransformationBLINATUMOMAB, IBRUTINIB, NIVOLUMABIts record names the diseaseClinicalTrials.gov

1 to 10 of 52

See all 52 trials

Comparisons in its trials5

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.

ComparisonGroup 1, with the outcomeGroup 2, with the outcomeParticipantsTrialSource
OFAR (Phase I) vs OFAR MTD (Phase II): serious adverse events8 of 1215 of 7890NCT00472849Trial comparisons
Pembrolizumab: Relapsed or Refractory Primary Mediastinal Large B-cell Lymphoma (rrPMBCL) vs Pembrolizumab: Relapsed or Refractory Richter Syndrome (rrRS): deaths from any cause30 of 5619 of 2480NCT02576990Trial comparisons
Pembrolizumab: Relapsed or Refractory Primary Mediastinal Large B-cell Lymphoma (rrPMBCL) vs Pembrolizumab: Relapsed or Refractory Richter Syndrome (rrRS): Number of Participants Who Discontinued Study Drug Due to an AE
6 of 534 of 2376NCT02576990Trial comparisons
Pembrolizumab: Relapsed or Refractory Primary Mediastinal Large B-cell Lymphoma (rrPMBCL) vs Pembrolizumab: Relapsed or Refractory Richter Syndrome (rrRS): Number of Participants Who Experienced an Adverse Event (AE)
50 of 5323 of 2376NCT02576990Trial comparisons
Pembrolizumab: Relapsed or Refractory Primary Mediastinal Large B-cell Lymphoma (rrPMBCL) vs Pembrolizumab: Relapsed or Refractory Richter Syndrome (rrRS): serious adverse events14 of 5315 of 2376NCT02576990Trial comparisons

Papers from its trials22

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMIDListed asTrials listing itSource
37130017RESULT1PubMed
32544980RESULT1PubMed
31609651RESULT1PubMed
25196783RESULT1PubMed
23265768RESULT1PubMed
41886642DERIVED1PubMed
41380698DERIVED1PubMed
39270702DERIVED1PubMed
38071379DERIVED1PubMed
37882668DERIVED1PubMed

Data dates

The date of each source this release read.

Open Targets
2026-09-18
Open Targets
Drug links and their checks, from Drugs and targets
2026-09-18
Drugs and targets
ClinicalTrials.gov, from Trials
2026-11-12
Trials
Comparisons, from Trial comparisons
2026-03-23
Trial comparisons
PubMed, for the checks
2026-11-06
PubMed
MeSH, for the checks
2026-11-10
MeSH

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_0002083

GET https://conductscience.org/api/diseases/v1/MONDO_0002083/drugs?page=2