Richter transformation
disease diseaseOn this page
Also known as chronic lymphocytic leukemia with Richter transformationchronic lymphocytic leukemia/small lymphocytic lymphoma with Richter transformationRichter syndromeRichter's syndromeRichter's transformation
Summary
Richter transformation (MONDO:0002083) is a disease and 66 clinical trials. Top therapeutic interventions include acalabrutinib, cyclophosphamide anhydrous, and obinutuzumab. A subtype of secondary neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Clinical trials: 66
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | Richter transformation |
| Mondo ID | MONDO:0002083 |
| DOID | DOID:1703 |
| ICD-11 | 1736108343 |
| NCIT | C35424 |
| SNOMED CT | 277550009 |
| UMLS | C0349631 |
| MedGen | 91159 |
| GARD | 0007578 |
| Is cancer (heuristic) | no |
Also known as: chronic lymphocytic leukemia with Richter transformation · chronic lymphocytic leukemia/small lymphocytic lymphoma with Richter transformation · Richter syndrome · Richter’s syndrome · Richter’s transformation
Disease family
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › secondary neoplasm › Richter transformation
Related subtypes (4): malignant spiradenoma, Pancoast syndrome, secondary malignant neoplasm, metastatic neoplasm
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 66.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 29 |
| PHASE1 | 21 |
| PHASE1/PHASE2 | 13 |
| Not specified | 2 |
| PHASE3 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT07220187 | PHASE3 | NOT_YET_RECRUITING | Adding Pirtobrutinib to the Usual Treatment for People With Newly Diagnosed Richter Transformation, The PIRAMID Trial |
| NCT02029443 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | ACP-196 (Acalabrutinib), a Novel Bruton Tyrosine Kinase (BTK) Inhibitor, for Treatment of Chronic Lymphocytic Leukemia, Richter’s Syndrome or Prolymphocytic Leukemia |
| NCT02846623 | PHASE2 | ACTIVE_NOT_RECRUITING | Atezolizumab, Obinutuzumab, and Venetoclax in Treating Patients With Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Relapsed or Refractory Richter Syndrome |
| NCT03054896 | PHASE2 | ACTIVE_NOT_RECRUITING | A Phase II Study of Venetoclax in Combination With Dose-adjusted EPOCH-R or R-CHOP for Patients With Richter’s Syndrome |
| NCT03162536 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | A Study of Nemtabrutinib (MK-1026) in Participants With Relapsed or Refractory Hematologic Malignancies (ARQ 531-101/MK-1026-001) |
| NCT03534323 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Duvelisib and Venetoclax in Relapsed or Refractory CLL or SLL or RS |
| NCT03899337 | PHASE2 | RECRUITING | A Trial of CHOP-R Therapy, With or Without Acalabrutinib, in Patients With Newly Diagnosed Richter’s Syndrome |
| NCT04271956 | PHASE2 | RECRUITING | Efficacy and Safety of Zanubrutinib Plus Tislelizumab Treatment with or Without Sonrotoclax for Patients with Richter Transformation |
| NCT04491370 | PHASE1/PHASE2 | RECRUITING | Autologous Stem Cell Transplant Followed by Polatuzumab Vedotin in Patients With B-cell Non-Hodgkin and Hodgkin Lymphoma |
| NCT04623541 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Safety and Efficacy Study of Epcoritamab in Subjects With Relapsed/Refractory Chronic Lymphocytic Leukemia and Richter’s Syndrome |
| NCT04679012 | PHASE2 | RECRUITING | Polatuzumab Vedotin in Combination With Chemotherapy in Subjects With Richter’s Transformation |
| NCT04792489 | PHASE2 | RECRUITING | DALY II USA/ MB-CART2019.1 for DLBCL |
| NCT05025800 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | ALX148, Rituximab and Lenalidomide for the Treatment of Indolent and Aggressive B-cell Non-Hodgkin Lymphoma |
| NCT05294731 | PHASE1/PHASE2 | RECRUITING | Treatment of Chinese Participants With B-Cell Malignancies With BGB-16673, a Bruton Tyrosine Kinase-Targeted Protein-Degrader |
| NCT05388006 | PHASE2 | RECRUITING | Acalabrutinib, Venetoclax and Durvalumab for the Treatment of Richter Transformation From Chronic Lymphocytic Leukemia or Small Lymphocytic Lymphoma |
| NCT05672173 | PHASE2 | RECRUITING | Lisocabtagene Maraleucel, Nivolumab and Ibrutinib for the Treatment of Richter’s Transformation |
| NCT06043674 | PHASE2 | RECRUITING | Phase 2 Study of Glofitamab Monotherapy & With Polatuzumab Vedotin, Pirtobrutinib, or Atezolizumab in Richter’s Transformation |
| NCT06186648 | PHASE2 | RECRUITING | Evaluation of Treatment by Glofitamab in Combination With Rituximab or Obinutuzumab Plus CHOP in Patients With RIchter Syndrome |
| NCT06863402 | PHASE2 | RECRUITING | Nemtabrutinib and Pembrolizumab for the Treatment of Richter Transformation, Diffuse Large B-cell Lymphoma Subtype |
| NCT06926205 | PHASE2 | RECRUITING | CHOP Plus Mosunetuzumab as First Line in Patients With Richter´s Syndrome: a Phase II Study of the Spanish Group of CLL |
| NCT06936943 | PHASE2 | RECRUITING | ONO-4538 Study in Patients With Richter’s Transformation |
| NCT00472849 | PHASE1/PHASE2 | COMPLETED | Oxaliplatin, Fludarabine, Cytarabine, and Rituximab in Patients With Richter’s Transformation and Leukemias |
| NCT01171378 | PHASE2 | COMPLETED | Study of Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone (CHOP) With Ofatumumab in Patients With Richter’s Syndrome |
| NCT01217749 | PHASE1/PHASE2 | COMPLETED | Efficacy and Safety Study of PCI-32765 Combine With Ofatumumab in CLL |
| NCT01629511 | PHASE1/PHASE2 | TERMINATED | Allogeneic Stem Cell Transplant for CLL |
| NCT02138786 | PHASE2 | TERMINATED | Selinexor in Initial or Refractory and/or Relapsed Richter’s Transformation |
| NCT02285244 | PHASE2 | WITHDRAWN | Sotrastaurin Acetate in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia, Small Lymphocytic Leukemia, Prolymphocytic Leukemia, or Richter’s Transformation |
| NCT02332980 | PHASE2 | COMPLETED | Pembrolizumab Alone or With Idelalisib or Ibrutinib in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia or Other Low-Grade B-Cell Non-Hodgkin Lymphomas |
| NCT02362035 | PHASE1/PHASE2 | COMPLETED | ACP-196 (Acalabrutinib) in Combination With Pembrolizumab, for Treatment of Hematologic Malignancies |
| NCT02378038 | PHASE2 | TERMINATED | PNT2258 for Treatment of Patients With Richter’s Transformation (Brighton) |
| NCT02420912 | PHASE2 | COMPLETED | Nivolumab and Ibrutinib in Treating Patients With Relapsed, Refractory, or High-Risk Untreated Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Richter Transformation |
| NCT02530515 | PHASE2 | COMPLETED | Ex Vivo-activated Autologous Lymph Node Lymphocytes in Treating Patients With Chronic Lymphocytic Leukemia |
| NCT02576990 | PHASE2 | COMPLETED | Study 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) |
| NCT03010358 | PHASE1/PHASE2 | COMPLETED | Entospletinib and Obinutuzumab in Treating Patients With Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Non-Hodgkin Lymphoma |
| NCT03121534 | PHASE2 | TERMINATED | Ibrutinib, Nivolumab and Blinatumomab in Richter Transformation |
| NCT03145480 | PHASE2 | TERMINATED | Study of Ibrutinib & Obinutuzumab With/Without CHOP for Richter’s Transformation or Richter’s Syndrome Patients |
| NCT03153514 | PHASE2 | TERMINATED | Obinutuzumab Containing Conditioning Regimen for Patients With Poor Risk CLL or Richter’s Transformation Requiring Allogeneic Stem Cell Transplantation |
| NCT03205046 | PHASE1/PHASE2 | TERMINATED | A Study of Acalabrutinib and Vistusertib in Subjects With Relapsed/Refractory B-cell Malignancies |
| NCT03931642 | PHASE2 | COMPLETED | BLINAtumomab After R-CHOP Debulking Therapy for Patients With Richter Transformation |
| NCT04305444 | PHASE2 | TERMINATED | Study of a Triple Combination Therapy, DTRM-555, in Patients With R/R CLL or R/R Non-Hodgkin’s Lymphomas |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| ACALABRUTINIB | 4 | 5 |
| CYCLOPHOSPHAMIDE ANHYDROUS | 4 | 5 |
| OBINUTUZUMAB | 4 | 4 |
| DUVELISIB | 4 | 3 |
| IBRUTINIB | 4 | 3 |
| PIRTOBRUTINIB | 4 | 3 |
| BLINATUMOMAB | 4 | 2 |
| OFATUMUMAB | 4 | 2 |
| CLOFARABINE | 4 | 1 |
| IDELALISIB | 4 | 1 |
| SELINEXOR | 4 | 1 |
| VENETOCLAX | 4 | 1 |
| NEMTABRUTINIB | 3 | 2 |
| ENTOSPLETINIB | 3 | 1 |
| SOTRASTAURIN ACETATE | 2 | 1 |
| VISTUSERTIB | 2 | 1 |
| CHEMBL4747506 | 0 | 5 |
| CHEMBL5187554 | 0 | 5 |
| CHEMBL5276925 | 0 | 5 |
| CHEMBL6165320 | 0 | 3 |
| CHEMBL3647964 | 0 | 2 |
| CHEMBL4466205 | 0 | 2 |
| CHEMBL4095008 | 0 | 1 |
| CHEMBL3970001 | 0 | 1 |