ReviewHematology. American Society of Hematology. Education Program2022
Treatment of Richter's syndrome.
Review in Hematology. American Society of Hematology. Education Program, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
20 citing papers in PubMed, 31 citations in OpenAlex.
- Blinatumomab after R-CHOP bridging therapy for patients with Richter transformation: a phase 2 multicentre trial.Nature communications · 2024Trial
- Precision Medicine in Non-Hodgkin Lymphoma: Advances in BTK Inhibition, CD30-Directed Antibody-Drug Conjugates, and HDAC-Mediated Epigenetic Therapy with Pirtobrutinib, Brentuximab Vedotin, and Belinostat.Journal of clinical medicine · 2026Review
- Identification of Central Genes in the Pathogenesis of Lipid Metabolism in Chronic Lymphocytic Leukaemia: A Multi-Omics Study Integrating Machine Learning and Mendelian Randomisation.Cell biochemistry and biophysics · 2026Article
- Molecular Pathogenesis and Targeted Treatment of Richter Transformation.Biomedicines · 2026Review
- A study comparing the efficacy of R-CHOP and R-DA-EPOCH regimens in young, high-risk treatment-naïve diffuse large B-cell lymphoma patients: a multicenter retrospective analysis.American journal of cancer research · 2026Article
- Chimeric Antigen Receptor T-Cell Therapy for Richter Transformation: A CIBMTR Analysis.Transplantation and cellular therapy · 2025Article
- p53/PD-L1 co-expression predicts poor prognosis in diffuse large B-cell lymphoma.Discover oncology · 2025Article
- JCMM Annual Review on Advances in Biotechnology for the Treatment of Haematological Malignancies: A Review of the Latest In-Patient Developments 2024-2025.Journal of cellular and molecular medicine · 2025Review
- Molecular Features Accompanying Richter's Transformation in Patients with Chronic Lymphocytic Leukemia.International journal of molecular sciences · 2025Review
- The Role of Artificial Intelligence and Radiomics in the Management of Lymphomas by PET/CT: The Clairvoyance in Clinic.Cancer management and research · 2025Review
- Lymphocytic Lymphoma Transforming into Hodgkin Lymphoma in Sub-Saharan Africa: Case Report and Literature Review.Hematology reports · 2024Article
- Allogeneic hematopoietic stem-cell transplantation for patients with Richter transformation: a retrospective study on behalf of the Chronic Malignancies Working Party of the EBMT.Bone marrow transplantation · 2024Article
- Molecular Subtypes and the Role of TP53 in Diffuse Large B-Cell Lymphoma and Richter Syndrome.Cancers · 2024Review
- A multicenter study of venetoclax-based treatment for patients with Richter transformation of chronic lymphocytic leukemia.Blood advances · 2024Article
- Decreased apoptotic priming and loss of BCL-2 dependence are functional hallmarks of Richter's syndrome.Cell death & disease · 2024Article
- Venetoclax plus dose-adjusted R-EPOCH (VR-DA-EPOCH) or G-EPOCH bridging to subsequent cellular therapy for the patients with transformed lymphoma a single center clinical experience.Annals of hematology · 2024Article
- Plasmablastic Lymphoma. A State-of-the-Art Review: Part 1-Epidemiology, Pathogenesis, Clinicopathologic Characteristics, Differential Diagnosis, Prognostic Factors, and Special Populations.Mediterranean journal of hematology and infectious diseases · 2024Review
- Ibrutinib combined with gemcitabine-vinorelbine for primary refractory non-Hodgkin lymphoma.Blood research · 2023Article
- Cytogenomic features of Richter transformation.Molecular cytogenetics · 2023Article
- Pirtobrutinib: a new hope for patients with BTK inhibitor-refractory lymphoproliferative disorders.Blood · 2023Article
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Richter's syndrome (RS) is an aggressive histologic transformation of chronic lymphocytic leukemia (CLL), most commonly to diffuse large B-cell lymphoma (DLBCL). Outcomes are generally poor, with complete remission (CR) rates of only about 20% and less than 20% long-term survival with chemoimmunotherapy (CIT). RS is biologically heterogeneous, and in 80% of patients with CLL who develop DLBCL, the disease is clonally related to the CLL. Clonally unrelated cases are genetically and immunologically distinct from clonally related DLBCL-RS, have more favorable responses to CIT, and are best treated as de novo DLBCL. Relatively favorable outcomes with CIT are also seen in patients who have never previously received treatment for CLL and who lack TP53 mutation or deletion. For the remaining patients, treatment on a clinical trial is optimal. Fortunately, numerous agents are now in clinical development that show encouraging results. Here we review clinical data for some of the most promising approaches. DLBCL-RS tumor cells frequently express programmed cell death 1 protein (PD-1), and several studies have demonstrated activity for PD-1 inhibitors, especially in combination with ibrutinib. The BCL2 inhibitor venetoclax in combination with R-EPOCH CIT achieved CR in 50% of patients, and a study of venetoclax-R-CHOP is ongoing. The noncovalent Bruton's tyrosine kinase inhibitor pirtobrutinib has achieved responses in approximately two-thirds of heavily pretreated patients and, given its favorable toxicity profile, appears ideally suited to combining with other active agents. Finally, we review available data for bispecific antibodies, antibody-drug conjugates, and chimeric antigen receptor T-cell therapy, which, after revolutionizing the treatment of DLBCL, are now being evaluated in RS.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.