Richter's Syndrome

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases
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Aliases & Classifications for Richter's Syndrome

MalaCards integrated aliases for Richter's Syndrome:

Name: Richter's Syndrome 11 14 71
Richter Syndrome 11 19 75
Richter Transformation 19


External Ids:

Disease Ontology 11 DOID:1703
NCIt 49 C35424
SNOMED-CT 68 277550009
ICD10 31 C91.1
UMLS 71 C0349631

Summaries for Richter's Syndrome

GARD: 19 Richter syndrome is a rare condition in which chronic lymphocytic leukemia (CLL) changes into a fast-growing type of lymphoma. Symptoms of Richter syndrome can include fever, loss of weight and muscle mass, abdominal pain, and enlargement of the lymph nodes, liver, and spleen. Laboratory results may show anemia and low platelet counts (which can lead to easy bleeding and bruising).

MalaCards based summary: Richter's Syndrome, also known as richter syndrome, is related to leukemia, chronic lymphocytic 2 and macroglobulinemia. An important gene associated with Richter's Syndrome is IGHV4-38-2 (Immunoglobulin Heavy Variable 4-38-2), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Nivolumab and Ofatumumab have been mentioned in the context of this disorder. Affiliated tissues include spleen, liver and t cells, and related phenotypes are Reduced mammosphere formation and cellular

Wikipedia: 75 Richter's transformation (RT), also known as Richter's syndrome, is the conversion of chronic... more...

Related Diseases for Richter's Syndrome

Diseases related to Richter's Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 298)
# Related Disease Score Top Affiliating Genes
1 leukemia, chronic lymphocytic 2 30.8 ZAP70 CD5 CD38 BCL2 ATM
2 macroglobulinemia 30.2 TP53 PAX5 BTK BCL2
3 immunodeficiency with hyper-igm, type 1 30.2 IGHV4-38-2 CD19 BTK
4 intravascular large b-cell lymphoma 30.1 BCL6 BCL2
5 skin carcinoma 30.0 TP53 NOTCH1 MYC BCL2
6 lymphoproliferative syndrome 29.8 TNFRSF8 TCL1A CD5 BCL6 BCL2
7 reticulosarcoma 29.8 TNFRSF8 PAX5 MYC BCL6 BCL2
8 lymphoma aids related 29.8 TCL1A MYC BCL6
9 pleural empyema 29.8 TNFRSF8 CD5 BCL6
10 anemia, autoimmune hemolytic 29.7 ZAP70 IGHV4-38-2 CD5 CD38 CD19 BTK
11 lymphoma, hodgkin, classic 29.7 ZAP70 TP53 TNFRSF8 PAX5 BCL6 BCL2
12 lymphosarcoma 29.7 TNFRSF8 BCL6 BCL2
13 myelodysplastic syndrome 29.6 TP53 MYC CD38 CD19 BCL2
14 chromosome 13q14 deletion syndrome 29.6 ZAP70 TP53 IGHV4-38-2 CD5 CD38 ATM
15 deficiency anemia 29.6 TP53 CD5 CD38 CD19 ATM
16 pancytopenia 29.5 TP53 CD5 CD22 CD19
17 b-cell prolymphocytic leukemia 29.5 IGHV4-38-2 CD79B CD5 CD22 CD19
18 adult t-cell leukemia/lymphoma 29.4 TP53 TNFRSF8 MYC CD5 BCL2
19 gastric lymphoma 29.2 TNFRSF8 IGHV4-38-2 CD5 BIRC3 BCL6 BCL2
20 angioimmunoblastic t-cell lymphoma 29.2 TNFRSF8 PAX5 IGHV4-38-2 CD5 BCL6
21 leukemia 29.2 ZAP70 TP53 TCL1B TCL1A NOTCH1 MYC
22 prolymphocytic leukemia 29.2 ZAP70 TP53 TCL1B TCL1A MYC CD5
23 bone lymphoma 29.1 TNFRSF8 PAX5 CD5 BCL6 BCL2
24 hematologic cancer 29.1 ZAP70 TNFRSF8 PAX5 NOTCH1 BCL2 ATM
25 diffuse large b-cell lymphoma 29.1 TNFRSF8 PAX5 MYC IGHV4-38-2 CD79B CD5
26 plasma cell neoplasm 29.1 TP53 PAX5 MYC IGHV4-38-2 CD38 CD19
27 t-cell prolymphocytic leukemia 29.1 TP53 TNFRSF8 TCL1B TCL1A IGHV4-38-2 CD5
28 waldenstroem's macroglobulinemia 29.0 ZAP70 PAX5 IGHV4-38-2 CD79B CD5 CD38
29 immune deficiency disease 29.0 ZAP70 TNFRSF8 CD5 CD38 BTK BCL6
30 follicular lymphoma 28.9 TP53 TCL1A PAX5 MYC CD5 BIRC3
31 plasmablastic lymphoma 28.9 TNFRSF8 PAX5 MYC IGHV4-38-2 CD38 BCL6
32 lymphatic system disease 28.8 TP53 TNFRSF8 MYC IGHV4-38-2 CD5 CD19
33 t-cell acute lymphoblastic leukemia 28.6 ZAP70 TNFRSF8 TCL1B TCL1A NOTCH1 MYC
34 burkitt lymphoma 28.6 TP53 TNFRSF8 TCL1A PAX5 MYC CD19
35 peripheral t-cell lymphoma 28.5 ZAP70 TP53 TNFRSF8 TCL1A PAX5 CD5
36 mature t-cell and nk-cell lymphoma 28.5 TP53 TNFRSF8 PAX5 MYC IGHV4-38-2 CD5
37 composite lymphoma 28.3 TNFRSF8 PAX5 IGHV4-38-2 CD5 CD22 BCL6
38 central nervous system lymphoma 28.3 TNFRSF8 PAX5 MYC IGHV4-38-2 CD79B CD5
39 lymphoma 28.3 TP53 TNFRSF8 TCL1B TCL1A PAX5 MYC
40 chronic lymphocytic leukemia/small lymphocytic lymphoma 27.8 ZAP70 TP53 TNFRSF8 PAX5 IGHV4-38-2 CD79B
41 b-cell lymphoma 27.7 ZAP70 TP53 TNFRSF8 TCL1A PAX5 MYC
42 leukemia, acute lymphoblastic 27.4 ZAP70 TP53 TCL1A PAX5 NOTCH1 MYC
43 marginal zone b-cell lymphoma 27.1 TNFRSF8 PAX5 MYC IGHV4-38-2 CD79B CD5
44 leukemia, chronic lymphocytic 27.0 ZAP70 TP53 TNFRSF8 TCL1B TCL1A RPS15
45 lymphoma, non-hodgkin, familial 26.9 TP53 TNFRSF8 TCL1A PAX5 NOTCH1 MYC
46 leukemia, acute myeloid 26.9 TP53 TNFRSF8 PAX5 NOTCH1 MYC IGHV4-38-2
47 mantle cell lymphoma 26.1 ZAP70 TP53 TNFRSF8 TCL1B TCL1A PAX5
48 kotzot-richter syndrome 11.2
49 solitary osseous plasmacytoma 10.3 IGHV4-38-2 CD38
50 congenital hypogammaglobulinemia 10.3 CD19 BTK

Graphical network of the top 20 diseases related to Richter's Syndrome:

Diseases related to Richter's Syndrome

Symptoms & Phenotypes for Richter's Syndrome

GenomeRNAi Phenotypes related to Richter's Syndrome according to GeneCards Suite gene sharing:

# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.17 ATM BCL6 CD22 CD5 MYC NOTCH1

MGI Mouse Phenotypes related to Richter's Syndrome:

# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.07 ATM BCL2 BCL6 BIRC3 BTK CD19
2 immune system MP:0005387 10.06 ATM BCL2 BCL6 BIRC3 BTK CD19
3 normal MP:0002873 10.02 BCL6 BIRC3 CD19 CD5 CD79B MYC
4 neoplasm MP:0002006 9.97 ATM BCL2 BTK CD19 MYC NOTCH1
5 hematopoietic system MP:0005397 9.86 ATM BCL2 BCL6 BIRC3 BTK CD19
6 integument MP:0010771 9.32 ATM BCL2 BCL6 BTK CD19 CD5

Drugs & Therapeutics for Richter's Syndrome

Drugs for Richter's Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 90)
# Name Status Phase Clinical Trials Cas Number PubChem Id
Nivolumab Approved Phase 2 946414-94-4
Ofatumumab Approved Phase 2 679818-59-8
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3 3680
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 3003 5743
Lenalidomide Approved Phase 1, Phase 2 191732-72-6 216326
Atezolizumab Approved, Investigational Phase 2 1380723-44-3
Durvalumab Approved, Investigational Phase 2 1428935-60-7
Zanubrutinib Approved, Investigational Phase 2 1691249-45-2 135565884
Fludarabine Approved Phase 2 75607-67-9, 21679-14-1 30751 657237
Pembrolizumab Approved Phase 1, Phase 2 1374853-91-4 254741536
Idelalisib Approved Phase 2 870281-82-6 11654566 11625818
Blinatumomab Approved, Investigational Phase 2 853426-35-4
Rituximab Approved Phase 2 174722-31-7
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
Prednisolone Approved, Vet_approved Phase 2 50-24-8 4894 5755
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5 1875
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 4159 6741
Venetoclax Approved, Investigational Phase 2 1257044-40-8 49846579
Etoposide Approved Phase 2 33419-42-0 36462
Valaciclovir Approved, Investigational Phase 2 124832-27-5, 124832-26-4 60773 135398742
Allopurinol Approved Phase 2 315-30-0 2094 135401907
Coal tar Approved Phase 2 8007-45-2
Acalabrutinib Approved, Investigational Phase 1, Phase 2 1420477-60-6 71226663 71226662
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
Obinutuzumab Approved, Investigational Phase 2 949142-50-1
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
Gemcitabine Approved Phase 1, Phase 2 95058-81-4, 122111-03-9 60750
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 5284616 6436030
Busulfan Approved, Investigational Phase 1, Phase 2 55-98-1 2478
Lenograstim Approved, Investigational Phase 1, Phase 2 135968-09-1
Levoleucovorin Approved, Experimental, Investigational Phase 1, Phase 2 68538-85-2, 58-05-9, 73951-54-9 149436 6006
Methotrexate Approved Phase 1, Phase 2 1959-05-2, 59-05-2 4112 126941
Tacrolimus Approved, Investigational Phase 1, Phase 2 104987-11-3 6473866 445643
Clofarabine Approved, Investigational Phase 1, Phase 2 123318-82-1 119182
D-Tyrosine Approved, Experimental, Investigational, Nutraceutical Phase 1, Phase 2 133585-56-5, 60-18-4, 556-02-5 1153 6057
Folic acid Approved, Nutraceutical, Vet_approved Phase 1, Phase 2 59-30-3 6037
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7 4897
41 Immune Checkpoint Inhibitors Phase 2
42 Antiemetics Phase 2
43 BB 1101 Phase 2
44 Gastrointestinal Agents Phase 2
45 Antibodies, Monoclonal Phase 2
Muromonab-CD3 Phase 1, Phase 2
47 Angiogenesis Inhibitors Phase 1, Phase 2
48 Immunoglobulins, Intravenous Phase 1, Phase 2
49 Immunoconjugates Phase 1, Phase 2
50 Immunoglobulin G Phase 2

Interventional clinical trials:

(show all 46)
# Name Status NCT ID Phase Drugs
1 A Phase I/II Study of Cloretazine in Patients With Refractory/Relapsed Chronic Lymphocytic Leukemia or Richter's Syndrome Completed NCT00304005 Phase 1, Phase 2 laromustine
2 Single Arm NCRI Feasibility Study of CHOP in Combination With Ofatumumab in Induction and Maintenance for Patients With Newly Diagnosed Richter's Syndrome Completed NCT01171378 Phase 2 Ofatumumab
3 A Phase II Study of Pembrolizumab (MK-3475) in Subjects With Relapsed or Refractory Primary Mediastinal Large B-cell Lymphoma (rrPMBCL) or Relapsed or Refractory Richter Syndrome (rrRS) Completed NCT02576990 Phase 2
4 A Phase I/II Study of Syk Inhibitor Entospletinib (GS-9973) in Combination With Obinutuzumab in Patients With Relapsed/Refractory Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) and B-Cell Malignancies Completed NCT03010358 Phase 1, Phase 2 Entospletinib
5 Nivolumab Combined With Ibrutinib for Relapsed, Refractory or High-Risk Untreated Patients With Chronic Lymphocytic Leukemia (CLL) Completed NCT02420912 Phase 2 Ibrutinib
6 Trial of Immune Reconstitution With Activated T-Cells in Patients With Chronic Lymphocytic Leukemia (CLL) Completed NCT02530515 Phase 2
7 A Phase II Study of Blinatumomab in Richter Transformation Completed NCT03121534 Phase 2 Blinatumomab;Dexamethasone
8 A Phase 1b/2, Open-Label, Safety and Efficacy Study of Epcoritamab (GEN3013; DuoBody®-CD3 X CD20) in Relapsed/Refractory Chronic Lymphocytic Leukemia and Richter's Syndrome Recruiting NCT04623541 Phase 1, Phase 2
9 Venetoclax Plus Dose-adjusted R-EPOCH or R-CHOP for Richter's Syndrome Recruiting NCT03054896 Phase 2 Venetoclax
10 A Phase I/II Study of Duvelisib and Venetoclax in Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia or Small Lymphocytic Lymphoma or Patients With Richter's Syndrome Recruiting NCT03534323 Phase 1, Phase 2 Duvelisib;Venetoclax
11 A Prospective, Phase-II Study to Evaluate the Efficacy and Safety of Obinutuzumab, Ibrutinib, and Venetoclax in Patients With Richter's Syndrome. GIVeRS Protocol: On Behalf of the Israeli CLL Study Group Recruiting NCT04939363 Phase 2
12 Phase II Study of Polatuzumab Vedotin in Combination With Chemotherapy in Subjects With Richter's Transformation Recruiting NCT04679012 Phase 2 Polatuzumab Vedotin;Rituximab;Etoposide;Prednisone;Cyclophosphamide;Hydroxydaunomycin
13 Safety and Tolerability of Myeloablative Conditioning and Autologous Stem Cell Transplantation Followed by Polatuzumab Vedotin (PV) Immunoconjugate Therapy in Patients With B-cell Non-Hodgkin and Hodgkin Lymphoma Recruiting NCT04491370 Phase 1, Phase 2 Polatuzumab vedotin
14 A Phase I/II Open Label, Single Center, Study of the Combination of ALX148, Rituximab and Lenalidomide in Patients With Indolent and Aggressive B-Cell Non-Hodgkin Lymphoma Recruiting NCT05025800 Phase 1, Phase 2 CD47 Antagonist ALX148;Lenalidomide
15 Phase II Study of a Combination Therapy of Acalabrutinib, Venetoclax and Durvalumab (MEDI4736) in Patients With Richter Transformation From Chronic Lymphocytic Leukemia (CLL) Recruiting NCT05388006 Phase 2 Acalabrutinib;Venetoclax
16 Atezolizumab (PD-L1 mAb) in Combination With Obinutuzumab and Venetoclax for Patients With Chronic Lymphocytic Leukemia and Richter Transformation Recruiting NCT02846623 Phase 2 Atezolizumab;Venetoclax
17 A Prospective, Open-label, Multicenter Phase-II Trial to Evaluate the Efficacy and Safety of Zanubrutinib (BGB-3111), a BTK Inhibitor, Plus Tislelizumab (BGB-A317), a PD1 Inhibitor, for Treatment of Patients With Richter Transformation Recruiting NCT04271956 Phase 2 Zanubrutinib
18 A Multi-Center, Open Label, Uncontrolled, Phase II Clinical Trial Evaluating the Safety and Efficacy of Venetoclax in Combination With Atezolizumab and Obinutuzumab in Richter Transformation of CLL Recruiting NCT04082897 Phase 2 Obinutuzumab 25 MG/ML [Gazyva];Atezolizumab 60 MG/ML [Tecentriq];Venetoclax Oral Tablet
19 A Phase 2, Open-Label, Multicenter, Basket Study Evaluating the Safety and Efficacy of Brexucabtagene Autoleucel in Adults With Rare B-cell Malignancies (ZUMA-25) Recruiting NCT05537766 Phase 2 Cyclophosphamide;Fludarabine
20 A Multicenter, Open-label, Phase 2 Basket Study to Evaluate the Safety and Efficacy of MK-2140 as a Monotherapy and in Combination in Participants With Aggressive and Indolent B-cell Malignancies Recruiting NCT05458297 Phase 2 Nemtabrutinib
21 A Phase 1/2, Multicenter, Open-label, and Dose-escalation Study of ACP-196 in Subjects With Chronic Lymphocytic Leukemia, Richter's Syndrome or Prolymphocytic Leukemia Active, not recruiting NCT02029443 Phase 1, Phase 2 Acalabrutinib
22 A Phase 1b/2 Proof-of-Concept Study of the Combination of ACP-196 (Acalabrutinib) and Pembrolizumab in Subjects With Hematologic Malignancies Active, not recruiting NCT02362035 Phase 1, Phase 2 Acalabrutinib;Pembrolizumab
23 A Phase II Study of Anti-PD-1 Antibody (MK-3475) in Relapsed/Refractory Chronic Lymphocytic Leukemia (CLL) and Other Low Grade B Cell Non-Hodgkin Lymphoma (NHL) Active, not recruiting NCT02332980 Phase 2 Ibrutinib;Idelalisib
24 BLINAtumomab After R-CHOP Debulking Therapy for Patients With Richter Transformation Active, not recruiting NCT03931642 Phase 2 RCHOP;Blinatumomab
25 A Phase II, Randomised Study of CHOP-R in Combination With Acalabrutinib Compared to CHOP-R in Patients With Newly Diagnosed Richter's Syndrome and a Platform for Initial Investigations Into Activity of Novel Treatments in Relapsed/Refractory and Newly Diagnosed Richter's Syndrome. Not yet recruiting NCT03899337 Phase 2 Acalabrutinib;Cyclophosphamide;Doxorubicin;Vincristine;Prednisolone;Rituximab
26 Time-limited Triplet Combination of Pirtobrutinib, Venetoclax, and Obinutuzumab for Patients With Treatment-naïve Chronic Lymphocytic Leukemia (CLL) or Richter Transformation (RT) Not yet recruiting NCT05536349 Phase 2 Pirtobrutinib;Obinutuzumab;Venetoclax;Valacyclovir;Allopurinol
27 A Multicenter Phase 2 Study of R-EPOCH in Combination With Ibrutinib for Patients With Classical Richter Transformation of Chronic Lymphocytic Leukemia Not yet recruiting NCT04992377 Phase 2 R-EPOCH in Combination With Ibrutinib
28 Two-arm Phase II Trial Exploring the Use of the Targeted Agents Ibrutinib and Obinutuzumab for the Treatment of Patients With a Diagnosis of Richter's Transformation (RT) or Richter's Syndrome (RS) Terminated NCT03145480 Phase 2 Obinutuzumab;Ibrutinib
29 A Phase 1/2 Proof-of-Concept Study of the Combination of Acalabrutinib and Vistusertib in Subjects With Relapsed/Refractory B-cell Malignancies Terminated NCT03205046 Phase 1, Phase 2 acalabrutinib;vistusertib
30 Clofarabine, Gemcitabine, and Busulfan Followed by Allogeneic Stem Cell Transplantation for Chronic Lymphocytic Leukemia (CLL) Terminated NCT01629511 Phase 1, Phase 2 Busulfan;Clofarabine;Gemcitabine;Methotrexate;Tacrolimus
31 A Phase 2 Feasibility Study of Sotrastaurin for Relapsed and Refractory CLL/SLL/PLL/RT Withdrawn NCT02285244 Phase 2 sotrastaurin acetate
32 Obinutuzumab, High Dose Methylprednisolone (HDMP), and Lenalidomide for the Treatment of Patients With Richter's Syndrome Completed NCT03113695 Phase 1 Obinutuzumab;lenalidomide;HDMP
33 A Phase 1, Open-Label, Multicentre, Non-Randomized Study to Assess the Safety, Tolerability, Pharmacokinetics and Preliminary Antitumor Activity of AZD4573, a Potent and Selective CDK9 Inhibitor, in Subjects With Relapsed or Refractory Haematological Malignancies Completed NCT03263637 Phase 1 AZD4573
34 Phase I Study of Ublituximab and Umbralisib in Combination With Targeted Immunotherapy in Patients With Relapsed-refractory Chronic Lymphocytic Leukemia (CLL) or Richter's Transformation (RT) of CLL Completed NCT02535286 Phase 1 Umbralisib
35 A Phase 1 Study of Lenalidomide Maintenance Following Allogeneic Hematopoietic Cell Transplantation in Patients With Select High Risk Hematological Malignancies Completed NCT01254578 Phase 1 Lenalidomide
36 An Open-label, Multicenter Phase 1 Dose Escalation Study to Characterize Safety, Tolerability, Preliminary Antitumor Activity, Pharmacokinetics, and Maximum Tolerated Dose of VIP152 in Subjects With Relapsed/Refractory Chronic Lymphocytic Leukemia or Richter Syndrome Recruiting NCT04978779 Phase 1 VIP152
37 A Phase I Study of PI3Kα,δ Inhibitor Copanlisib in Combination With PD-1 Antagonist Nivolumab in Patients With Transformed Chronic Lymphocytic Leukemia (Richter's Transformation) or Non-Hodgkin Lymphoma Recruiting NCT03884998 Phase 1 Copanlisib
38 Ipilimumab Combined With Ibrutinib and Nivolumab for Patients With Chronic Lymphocytic Leukemia (CLL) and Richter Transformation (RT) Recruiting NCT04781855 Phase 1 Ibrutinib
39 A Multicenter, Open-label, Phase 1 Study Evaluating the Safety and Tolerability of HMPL-760 in Patients With Previously Treated Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) or Other Non-Hodgkin Lymphoma (NHL) Recruiting NCT05176691 Phase 1 HMPL-760
40 A Phase 1/1b Study Evaluating the Safety, Pharmacokinetics, and Preliminary Efficacy of LP-118 in Subjects With Relapsed or Refractory Hematological Malignancies Recruiting NCT04771572 Phase 1 LP-118
41 A Phase 1a, Dose Escalation, Safety and Tolerability Study of NX-1607, a Casitas B-lineage Lymphoma Proto-oncogene (CBL-B) Inhibitor, in Adults With Advanced Malignancies, With Phase 1b Expansion in Select Tumor Types Recruiting NCT05107674 Phase 1 NX-1607
42 A Phase I Study of Duvelisib in Combination With Nivolumab for Patients With Richter's Syndrome and Transformed Follicular Lymphoma Active, not recruiting NCT03892044 Phase 1 Duvelisib
43 A Pilot Phase I Study of Atezolizumab (MPDL3280A) in Combination With Immunogenic Chemotherapy (Gemcitabine-Oxaliplatin) and Rituximab for Transformed Diffuse Large B-Cell Lymphoma Active, not recruiting NCT03321643 Phase 1 Atezolizumab;Gemcitabine;Oxaliplatin
44 A Phase I Study of Pevonedistat (MLN4924, TAK924) in Combination With Ibrutinib in Patients With Relapsed/Refractory Chronic Lymphocytic Leukemia and Non-Hodgkin Lymphoma Active, not recruiting NCT03479268 Phase 1 Ibrutinib;Pevonedistat
45 A Phase 1 Dose-Escalation and Cohort-Expansion Study of VLS-101 in Subjects With Hematological Malignancies (waveLINE-001) Active, not recruiting NCT03833180 Phase 1 Zilovertamab vedotin
46 Genomic and Proteomic Study of Richter Syndrome Unknown status NCT03619512

Search NIH Clinical Center for Richter's Syndrome

Genetic Tests for Richter's Syndrome

Anatomical Context for Richter's Syndrome

Organs/tissues related to Richter's Syndrome:

MalaCards : Spleen, Liver, T Cells, Bone Marrow, B Cells, Lymph Node, Bone

Publications for Richter's Syndrome

Articles related to Richter's Syndrome:

(show top 50) (show all 728)
# Title Authors PMID Year
Necrotizing herpes simplex lymphadenitis in a patient with chronic lymphocytic leukaemia/small lymphocytic lymphoma, clinically masquerading as Richter transformation. 62
35811089 2022
Long-term follow-up and future direction on the management of chronic lymphocytic leukemia/small lymphocytic leukemia. 62
35635742 2022
Macrophage- and BCR-derived but not TLR-derived signals support the growth of CLL and Richter syndrome murine models in vivo. 62
36084319 2022
In vivo modeling of CLL transformation to Richter's syndrome reveals convergent evolutionary paths and therapeutic vulnerabilities. 62
36468984 2022
Minimal residual disease-driven treatment intensification with sequential addition of ibrutinib to venetoclax in R/R CLL. 62
35921541 2022
Mitochondrial DNA mutations as natural barcodes for lineage tracing of murine tumor models. 62
36469010 2022
Richter transformation in Chronic Lymphocytic Leukemia. 62
36433773 2022
Insights from a patient with chronic lymphocytic leukemia complicating ALK+ anaplastic large cell lymphoma. 62
36457588 2022
Deregulation and epigenetic modification of BCL2-family genes cause resistance to venetoclax in hematologic malignancies. 62
35704690 2022
An experience with ibrutinib monotherapy for Richter's syndrome isolated in the central nervous system. 62
36436931 2022
TP53/PLCG2-mutated diffuse large B-cell lymphoma richter transformation (DLBCL-RT) of CLL with unusual CD2 and PD-1 expression. 62
35719104 2022
Richter transformation to aggressive plasmablastic neoplasm related to selection of a BTK-mutated clone in a patient with CLL/SLL treated by ibrutinib. 62
36336991 2022
A Phase 2 Study of Nivolumab Combined with Ibrutinib in Patients with Diffuse Large B-cell Richter Transformation of CLL. 62
36287248 2022
Early Clones Expand in Richter Transformation of Chronic Lymphocytic Leukemia. 62
35984230 2022
Long-term effect of venetoclax therapy in a patient with the transformation of chronic lymphocytic leukemia into Richter's syndrome. 62
36299674 2022
Hodgkin's variant of Richter transformation during ibrutinib therapy: A case report and review of the literature. 62
36225619 2022
Richter Syndrome: From Molecular Pathogenesis to Druggable Targets. 62
36230566 2022
Emerging Therapies for the Management of Richter Transformation. 62
36130148 2022
Distinct immune-response profile of Richter transformation chronic lymphocytic leukemia (CLL), defined by high expression of PD1, LAG3, TIM3 and IL-10. 62
36102789 2022
Clonally unrelated Richter syndrome are truly de novo diffuse large B-cell lymphomas with a mutational profile reminiscent of clonally related Richter syndrome. 62
35829664 2022
Clinical outcomes in patients with chronic lymphocytic leukemia with disease progression on ibrutinib. 62
36050317 2022
Anti-CD37 α-amanitin-conjugated antibodies as potential therapeutic weapons for Richter syndrome. 62
35914223 2022
A phase two study of high dose blinatumomab in Richter's syndrome. 62
35941212 2022
The clonal evolution of Richter transformation cells uncovers therapeutic vulnerabilities. 62
35953722 2022
Richter's syndrome in central nervous system with MYD88L265P and CD79b mutation responded well to ibrutinib containing chemotherapy: a case report and review of the literature. 62
35484253 2022
Detection of early seeding of Richter transformation in chronic lymphocytic leukemia. 62
35953718 2022
Outcomes of Hodgkin variant Richter transformation in chronic lymphocytic leukaemia and small lymphocytic lymphoma in British Columbia. 62
35567407 2022
Hodgkin Lymphoma and Hairy Cell Leukemia Arising from Chronic Lymphocytic Leukemia: Case Reports and Literature Review. 62
36012912 2022
Artificial intelligence-assisted mapping of proliferation centers allows the distinction of accelerated phase from large cell transformation in chronic lymphocytic leukemia. 62
35132162 2022
Unusual Illustration of Richter Transformation in Chronic Lymphocytic Leukemia on FDG PET/CT. 62
35307722 2022
Selinexor Combined with Ibrutinib Demonstrates Tolerability and Safety in Advanced B-Cell Malignancies: A Phase I Study. 62
35608822 2022
The Pt(S-pr-thiosal)2 and BCL1 Leukemia Lymphoma: Antitumor Activity In Vitro and In Vivo. 62
35897737 2022
Aberrant expression of lymphoid enhancer-binding factor 1 in Hodgkin lymphoma. 62
35421421 2022
Richter Transformation: Clinical Manifestations, Evaluation, and Management. 62
35910504 2022
SOHO State of the Art Updates and Next Questions | Management of Most Difficult Cases of Chronic Lymphocytic Leukemia: Relapse After Both BTK and BCL2 Inhibition and Richter Transformation. 62
35577753 2022
Dual peaks on flow cytometric DNA ploidy analysis in chronic lymphocytic leukemia with Richter transformation. 62
35560867 2022
An Approach to Diagnosis of Richter Transformation in Chronic Lymphocytic Leukemia. 62
35910501 2022
Distinct Chemokine Receptor Expression Profiles in De Novo DLBCL, Transformed Follicular Lymphoma, Richter's Trans-Formed DLBCL and Germinal Center B-Cells. 62
35887224 2022
[Chronic lymphocytic leukemia]. 62
35184869 2022
Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma With Secondary Acquisition of t(11;14)(q13;q32)/CCND1-IGH: A Rare Variant Of Richter Transformation to Mantle Cell Lymphoma. 62
34840090 2022
Chronic Lymphocytic Leukemia Progression Diagnosis with Intrinsic Cellular Patterns via Unsupervised Clustering. 62
35626003 2022
Infection as a Differential Diagnosis of Solid Retroperitoneal Masses: A Case Series and Review of the Literature. 62
35663681 2022
Differential transcriptomic profiling in ibrutinib-naïve versus ibrutinib-resistant Richter syndrome. 62
34806797 2022
The complex karyotype landscape in chronic lymphocytic leukemia allows the refinement of the risk of Richter syndrome transformation. 62
34092056 2022
Nodal hodgkin richter transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma. 62
35435414 2022
Novel Approaches for the Treatment of Patients with Richter's Syndrome. 62
35294723 2022
Clinical outcome of allogeneic stem cell transplantation in patients with B-cell lymphoid malignancies following treatment with targeted small molecule inhibitors. 62
35225133 2022
Cellular Therapy Advances in Chronic Lymphocytic Leukemia and Richter's Syndrome. 62
34991902 2022
Venetoclax plus dose-adjusted R-EPOCH for Richter syndrome. 62
34788401 2022
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Variations for Richter's Syndrome

Expression for Richter's Syndrome

Search GEO for disease gene expression data for Richter's Syndrome.

Pathways for Richter's Syndrome

Pathways related to Richter's Syndrome according to GeneCards Suite gene sharing:

(show all 29)
# Super pathways Score Top Affiliating Genes
Show member pathways
13.88 ZAP70 TP53 TNFRSF8 MYC CD79B CD22
Show member pathways
Show member pathways
12.85 TP53 TCL1B TCL1A MYC CD19 BCL2
Show member pathways
5 12.32 ZAP70 TNFRSF8 PAX5 CD38 CD19 BTK
Show member pathways
12.24 CD79B CD22 CD19 BTK BCL6
Show member pathways
12.19 ZAP70 CD22 CD19 BTK BCL6
8 12.04 TP53 MYC BCL2 ATM
9 11.88 TP53 MYC BCL6 BCL2
Show member pathways
11.84 TP53 BCL6 ATM
11 11.84 BCL2 BCL6 MYC NOTCH1
12 11.81 TP53 MYC BIRC3 BCL2
13 11.73 TP53 MYC BCL6 BCL2 ATM
14 11.72 TP53 BCL6 ATM
15 11.7 PAX5 MYC BIRC3 BCL2
Show member pathways
17 11.62 TP53 MYC ATM
18 11.61 PAX5 CD22 CD19 BCL6
19 11.57 TP53 NOTCH1 ATM
20 11.51 BIRC3 MYC TP53
21 11.4 TP53 MYC ATM
Show member pathways
11.32 CD79B CD22 CD19 BTK
23 11.28 ATM BCL2 MYC TP53
24 11.17 CD79B CD22 CD19 BTK BCL6
25 11.16 NOTCH1 CD79B CD5 CD38 CD22 CD19
Show member pathways
10.76 TP53 MYC BCL2 ATM
27 10.63 TP53 MYC
28 10.38 TP53 ATM
29 10.32 ZAP70 TP53 BCL2

GO Terms for Richter's Syndrome

Cellular components related to Richter's Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein-containing complex GO:0032991 9.47 TP53 TCL1B TCL1A MYC CD19 BIRC3

Biological processes related to Richter's Syndrome according to GeneCards Suite gene sharing:

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of apoptotic process GO:0043066 10.07 TP53 TCL1A MYC CD38 BIRC3 BCL6
2 cellular response to hypoxia GO:0071456 10.06 TP53 NOTCH1 MYC BCL2
3 B cell proliferation GO:0042100 10.02 CD38 BCL6 BCL2
4 immune system process GO:0002376 9.95 ATM BCL6 BTK CD19 CD79B ZAP70
5 B cell activation GO:0042113 9.91 ZAP70 CD22 BTK
6 positive regulation of apoptotic process GO:0043065 9.9 ATM BCL2 BCL6 NOTCH1 TNFRSF8 TP53
7 response to gamma radiation GO:0010332 9.88 TP53 MYC BCL2
8 B cell differentiation GO:0030183 9.85 BCL2 BCL6 CD79B PAX5
9 mesenchymal cell development GO:0014031 9.84 NOTCH1 BCL2
10 B cell lineage commitment GO:0002326 9.8 TP53 BCL2
11 T cell lineage commitment GO:0002360 9.69 TP53 BCL2
12 positive regulation of B cell proliferation GO:0030890 9.56 CD38 BTK BCL6 BCL2
13 regulation of programmed cell death GO:0043067 9.52 BIRC3 BCL2
14 negative regulation of leukocyte proliferation GO:0070664 9.5 BTK BCL6
15 B cell receptor signaling pathway GO:0050853 9.36 IGHV4-38-2 CD79B CD38 CD19 BTK BCL2

Molecular functions related to Richter's Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transcription regulator activator activity GO:0140537 8.8 NOTCH1 MYC

Sources for Richter's Syndrome

8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
27 GO
28 GTR
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
36 LifeMap
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
70 Tocris
72 UMLS via Orphanet
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