MCID: RCH001
MIFTS: 50

Richter's Syndrome

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases

Aliases & Classifications for Richter's Syndrome

MalaCards integrated aliases for Richter's Syndrome:

Name: Richter's Syndrome 12 15 73
Richter Syndrome 12 76 53
Richter Transformation 53

Classifications:



External Ids:

Disease Ontology 12 DOID:1703
ICD10 33 C91.1
NCIt 50 C35424
UMLS 73 C0349631

Summaries for Richter's Syndrome

NIH Rare Diseases : 53 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 and b-cell lymphomas. An important gene associated with Richter's Syndrome is CD5 (CD5 Molecule), and among its related pathways/superpathways are TCR Signaling (Qiagen) and PI3K-Akt signaling pathway. The drugs Fludarabine and Mycophenolic acid have been mentioned in the context of this disorder. Affiliated tissues include b cells, lymph node and liver, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Wikipedia : 76 Richter''s syndrome (RS), also known as Richter''s transformation, is a transformation of B cell 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 78)
# Related Disease Score Top Affiliating Genes
1 leukemia, chronic lymphocytic 30.6 ATM CD38 CD5 NOTCH1 TP53
2 b-cell lymphomas 30.5 CD5 IRF4 MYC TP53
3 diffuse large b-cell lymphoma 30.4 CD5 IRF4 MYC TP53
4 lymphocytic leukemia 30.4 ATM CD38 CD5 MYC NOTCH1 TP53
5 cll/sll 30.0 ATM CD38 CD5 TP53
6 mantle cell lymphoma 29.8 ATM CD5 MYC TP53
7 plasmablastic lymphoma 29.8 CD38 MYC NOTCH1
8 prolymphocytic leukemia 29.6 ATM CD38 CD5 MYC
9 lymphoma, non-hodgkin, familial 29.3 ATM CD38 CD5 IRF4 MYC NOTCH1
10 kotzot-richter syndrome 12.1
11 leukemia, chronic lymphocytic 2 10.6
12 leukemia, b-cell, chronic 10.6
13 leukemia 10.5
14 lymphoma 10.5
15 colon lymphoma 10.2 CD5 IRF4
16 t-cell adult acute lymphocytic leukemia 10.2 CD5 IRF4
17 waldenstroem's macroglobulinemia 10.2 CD38 CD5
18 plasma protein metabolism disease 10.2 CD38 CD5
19 splenic diffuse red pulp small b-cell lymphoma 10.2 NOTCH1 TP53
20 primary mediastinal large b-cell lymphoma 10.2 IRF4 MYC
21 lymphoma, hodgkin, classic 10.2
22 invasive bladder transitional cell carcinoma 10.2 NOTCH1 TP53
23 central nervous system hematologic cancer 10.2 IRF4 TP53
24 primary effusion lymphoma 10.1 IRF4 MYC
25 megaesophagus 10.1 MYC TP53
26 spinal cord astrocytoma 10.1 NOTCH1 TP53
27 hypertrichosis universalis congenita, ambras type 10.1 MYC TP53
28 suppressor of tumorigenicity 3 10.1 MYC TP53
29 necrotizing sialometaplasia 10.1 CD5 TP53
30 synchronous bilateral breast carcinoma 10.1 ATM TP53
31 adult lymphoma 10.1 CD5 MYC
32 uterine corpus cancer 10.1 MYC TP53
33 herpes simplex 10.1
34 anal squamous cell carcinoma 10.1 MLH1 TP53
35 myxosarcoma 10.1 ATM TP53
36 plasma cell neoplasm 10.1 CD38 MYC
37 plasmacytoma 10.1 CD38 IRF4 MYC
38 cerebral primitive neuroectodermal tumor 10.1 MYC TP53
39 mature b-cell neoplasm 10.1 CD38 MYC TP53
40 medullomyoblastoma 10.1 MYC TP53
41 acquired immunodeficiency syndrome 10.1 CD38 MYC TP53
42 childhood medulloblastoma 10.0 MYC TP53
43 lymphatic system cancer 10.0 CD5 MYC
44 lymphadenitis 10.0
45 merkel cell carcinoma 10.0
46 pancreas adenocarcinoma 10.0 MYC NOTCH1 TP53
47 colonic disease 10.0 MLH1 MYC TP53
48 fallopian tube carcinoma 10.0 MYC TP53
49 intestinal disease 10.0 MLH1 MYC TP53
50 reproductive system disease 10.0 MLH1 MYC TP53

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:

26 (show top 50) (show all 56)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-105 10.58 ATM
2 Increased shRNA abundance (Z-score > 2) GR00366-A-11 10.58 ATM
3 Increased shRNA abundance (Z-score > 2) GR00366-A-114 10.58 ATM IRF4 THBS1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-120 10.58 NOTCH1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-122 10.58 ATM
6 Increased shRNA abundance (Z-score > 2) GR00366-A-124 10.58 MLH1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-130 10.58 MLH1
8 Increased shRNA abundance (Z-score > 2) GR00366-A-137 10.58 POLB
9 Increased shRNA abundance (Z-score > 2) GR00366-A-138 10.58 MLH1 NOTCH1 POLB
10 Increased shRNA abundance (Z-score > 2) GR00366-A-147 10.58 NOTCH1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-149 10.58 ATM
12 Increased shRNA abundance (Z-score > 2) GR00366-A-151 10.58 ATM
13 Increased shRNA abundance (Z-score > 2) GR00366-A-152 10.58 IRF4
14 Increased shRNA abundance (Z-score > 2) GR00366-A-159 10.58 ATM THBS1
15 Increased shRNA abundance (Z-score > 2) GR00366-A-16 10.58 ATM
16 Increased shRNA abundance (Z-score > 2) GR00366-A-161 10.58 NOTCH1 POLB
17 Increased shRNA abundance (Z-score > 2) GR00366-A-166 10.58 THBS1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-169 10.58 IRF4 THBS1
19 Increased shRNA abundance (Z-score > 2) GR00366-A-195 10.58 MLH1
20 Increased shRNA abundance (Z-score > 2) GR00366-A-2 10.58 IRF4
21 Increased shRNA abundance (Z-score > 2) GR00366-A-205 10.58 NOTCH1
22 Increased shRNA abundance (Z-score > 2) GR00366-A-207 10.58 POLB
23 Increased shRNA abundance (Z-score > 2) GR00366-A-214 10.58 NOTCH1 ATM
24 Increased shRNA abundance (Z-score > 2) GR00366-A-215 10.58 ATM
25 Increased shRNA abundance (Z-score > 2) GR00366-A-23 10.58 POLB
26 Increased shRNA abundance (Z-score > 2) GR00366-A-41 10.58 ATM
27 Increased shRNA abundance (Z-score > 2) GR00366-A-43 10.58 POLB
28 Increased shRNA abundance (Z-score > 2) GR00366-A-50 10.58 THBS1
29 Increased shRNA abundance (Z-score > 2) GR00366-A-52 10.58 POLB
30 Increased shRNA abundance (Z-score > 2) GR00366-A-54 10.58 POLB
31 Increased shRNA abundance (Z-score > 2) GR00366-A-56 10.58 IRF4
32 Increased shRNA abundance (Z-score > 2) GR00366-A-6 10.58 NOTCH1
33 Increased shRNA abundance (Z-score > 2) GR00366-A-73 10.58 NOTCH1
34 Increased shRNA abundance (Z-score > 2) GR00366-A-74 10.58 MLH1
35 Increased shRNA abundance (Z-score > 2) GR00366-A-78 10.58 MLH1 THBS1
36 Increased shRNA abundance (Z-score > 2) GR00366-A-8 10.58 ATM
37 Increased shRNA abundance (Z-score > 2) GR00366-A-81 10.58 ATM THBS1
38 Increased shRNA abundance (Z-score > 2) GR00366-A-82 10.58 POLB IRF4
39 Increased shRNA abundance (Z-score > 2) GR00366-A-85 10.58 ATM
40 Increased shRNA abundance (Z-score > 2) GR00366-A-88 10.58 MLH1
41 Increased shRNA abundance (Z-score > 2) GR00366-A-93 10.58 THBS1
42 Increased shRNA abundance (Z-score > 2) GR00366-A-99 10.58 IRF4
43 Decreased shRNA abundance (Z-score < -2) GR00366-A-139 10.13 ATM NOTCH1
44 Decreased shRNA abundance (Z-score < -2) GR00366-A-142 10.13 THBS1
45 Decreased shRNA abundance (Z-score < -2) GR00366-A-160 10.13 ATM
46 Decreased shRNA abundance (Z-score < -2) GR00366-A-187 10.13 THBS1
47 Decreased shRNA abundance (Z-score < -2) GR00366-A-20 10.13 THBS1
48 Decreased shRNA abundance (Z-score < -2) GR00366-A-28 10.13 NOTCH1
49 Decreased shRNA abundance (Z-score < -2) GR00366-A-65 10.13 ATM NOTCH1 THBS1
50 Decreased shRNA abundance (Z-score < -2) GR00366-A-75 10.13 NOTCH1

MGI Mouse Phenotypes related to Richter's Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.11 ATM CD38 IRF4 MLH1 MYC NOTCH1
2 hematopoietic system MP:0005397 10.02 ATM CD38 CD5 IRF4 MLH1 MYC
3 endocrine/exocrine gland MP:0005379 10.01 ATM CD38 MLH1 MYC NOTCH1 POLB
4 digestive/alimentary MP:0005381 9.98 IL19 MLH1 MYC NOTCH1 POLB THBS1
5 growth/size/body region MP:0005378 9.97 ATM IL19 IRF4 MYC NOTCH1 POLB
6 homeostasis/metabolism MP:0005376 9.96 ATM CD38 IL19 IRF4 MLH1 MYC
7 immune system MP:0005387 9.9 ATM CD38 CD5 IL19 IRF4 MLH1
8 integument MP:0010771 9.56 ATM CD5 MLH1 MYC NOTCH1 POLB
9 neoplasm MP:0002006 9.17 ATM IRF4 MLH1 MYC NOTCH1 THBS1

Drugs & Therapeutics for Richter's Syndrome

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

(show top 50) (show all 104)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fludarabine Approved Phase 3,Phase 1,Phase 2 75607-67-9, 21679-14-1 30751
2
Mycophenolic acid Approved Phase 3,Phase 2,Phase 1 24280-93-1 446541
3
Everolimus Approved Phase 3,Phase 1,Phase 2 159351-69-6 6442177
4
Sirolimus Approved, Investigational Phase 3,Phase 1,Phase 2 53123-88-9 46835353 6436030 5284616
5
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 2 22916-47-8 4189
6 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1
7 Antitubercular Agents Phase 3,Phase 2,Phase 1
8 Vidarabine Phosphate Phase 3,Phase 2,Phase 1
9 Calcineurin Inhibitors Phase 3,Phase 2,Phase 1
10 Antimetabolites, Antineoplastic Phase 3,Phase 1,Phase 2
11 Immunosuppressive Agents Phase 3,Phase 1,Phase 2
12 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
13 Antifungal Agents Phase 3,Phase 2
14 Antimetabolites Phase 3,Phase 1,Phase 2
15 Immunologic Factors Phase 3,Phase 2,Phase 1
16 Antirheumatic Agents Phase 3,Phase 1,Phase 2
17 Cyclosporins Phase 3,Phase 2
18 Anti-Infective Agents Phase 3,Phase 1,Phase 2
19 Dermatologic Agents Phase 3,Phase 2,Phase 1
20
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
21
Cyclophosphamide Approved, Investigational Phase 2,Phase 1 6055-19-2, 50-18-0 2907
22
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
23
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
24
Ofatumumab Approved Phase 2,Phase 1 679818-59-8 6918251
25
rituximab Approved Phase 1, Phase 2,Phase 2 174722-31-7 10201696
26
Vidarabine Approved, Investigational Phase 1, Phase 2,Phase 2 24356-66-9 32326 21704
27
Oxaliplatin Approved, Investigational Phase 1, Phase 2,Phase 2 61825-94-3 43805 6857599 5310940 9887054
28
Cytarabine Approved, Investigational Phase 1, Phase 2,Phase 2 147-94-4 6253
29
Lenalidomide Approved Phase 2,Phase 1 191732-72-6 216326
30
Bevacizumab Approved, Investigational Phase 2 216974-75-3
31
Chlorambucil Approved Phase 2 305-03-3 2708
32
Methylprednisolone Approved, Vet_approved Phase 2,Phase 1 83-43-2 6741
33
Prednisolone phosphate Approved, Vet_approved Phase 2,Phase 1 302-25-0
34
Prednisolone Approved, Vet_approved Phase 2,Phase 1 50-24-8 5755
35
Methylprednisolone hemisuccinate Approved Phase 2,Phase 1 2921-57-5
36
alemtuzumab Approved, Investigational Phase 2 216503-57-0
37
Venetoclax Approved, Investigational Phase 2 1257044-40-8 49846579
38
Etoposide Approved Phase 2 33419-42-0 36462
39
Obinutuzumab Approved, Investigational Phase 2,Phase 1 949142-50-1
40 Blinatumomab Approved, Investigational Phase 2 853426-35-4
41
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
42
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
43
Pembrolizumab Approved Phase 1, Phase 2,Phase 2 1374853-91-4
44
Idelalisib Approved Phase 2 870281-82-6
45
Gemcitabine Approved Phase 1, Phase 2 95058-81-4 60750
46
Nivolumab Approved Phase 1, Phase 2 946414-94-4
47
leucovorin Approved Phase 2,Phase 1 58-05-9 6006 143
48
Tacrolimus Approved, Investigational Phase 2,Phase 1 104987-11-3 445643 439492
49
Methotrexate Approved Phase 2,Phase 1 59-05-2, 1959-05-2 126941
50
Busulfan Approved, Investigational Phase 2,Phase 1 55-98-1 2478

Interventional clinical trials:

(show top 50) (show all 62)
# Name Status NCT ID Phase Drugs
1 Graft-Versus-Host Disease Prophylaxis in Treating Patients With Hematologic Malignancies Undergoing Unrelated Donor Peripheral Blood Stem Cell Transplant Completed NCT01231412 Phase 3 Cyclosporine;Fludarabine Phosphate;Mycophenolate Mofetil;Sirolimus
2 Elevate CLL R/R: Study of Acalabrutinib (ACP-196) Versus Ibrutinib in Previously Treated Subjects With High Risk Chronic Lymphocytic Leukemia Active, not recruiting NCT02477696 Phase 3 ACP-196;ibrutinib
3 ACP-196 (Acalabrutinib), a Novel Bruton Tyrosine Kinase (Btk) Inhibitor, for Treatment of Chronic Lymphocytic Leukemia Unknown status NCT02029443 Phase 1, Phase 2 acalabrutinib
4 Study of Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone (CHOP) With Ofatumumab in Patients With Richter's Syndrome Completed NCT01171378 Phase 2 Ofatumumab
5 VNP40101M in Treating Patients With Richter Syndrome or Refractory or Relapsed Chronic Lymphocytic Leukemia or Other Lymphoproliferative Disorders Completed NCT00304005 Phase 1, Phase 2 laromustine
6 Oxaliplatin, Fludarabine, Cytarabine, and Rituximab in Patients With Richter's Transformation and Leukemias Completed NCT00472849 Phase 1, Phase 2 Oxaliplatin;Fludarabine;Cytarabine;Rituximab;Pegfilgrastim
7 Oxaliplatin, Fludarabine, Cytarabine and Rituximab in Richter's Syndrome, Refractory CLL and PLL Completed NCT00452374 Phase 1, Phase 2 Cytarabine;Fludarabine;Oxaliplatin;Rituximab
8 Combination Chemotherapy and Rituximab in Treating Patients With Chronic Lymphocytic Leukemia (CLL) That Did Not Respond to Fludarabine, CLL With Autoimmune Haemolytic Anemia (AIHA) or Richter's Transformation (RT) Completed NCT00309881 Phase 2 cyclophosphamide;doxorubicin hydrochloride;prednisone;vincristine sulfate
9 Efficacy and Safety Study of PCI-32765 Combine With Ofatumumab in CLL Completed NCT01217749 Phase 1, Phase 2 PCI-32765;ofatumumab
10 Fludarabine, Cyclophosphamide, and Rituximab - High Dose Frontline Completed NCT00794820 Phase 2 Fludarabine Phosphate;Cyclophosphamide;Rituximab
11 Ex Vivo-activated Autologous Lymph Node Lymphocytes in Treating Patients With Chronic Lymphocytic Leukemia Completed NCT02530515 Phase 2
12 Bevacizumab in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia Completed NCT00290810 Phase 2
13 Fludarabine Phosphate and Total-Body Irradiation Before Donor Peripheral Blood Stem Cell Transplant in Treating Patients With Chronic Lymphocytic Leukemia or Small Lymphocytic Leukemia Completed NCT00060424 Phase 2 Cyclosporine;Fludarabine Phosphate;Mycophenolate Mofetil
14 A Study of MabThera (Rituximab) Plus Chlorambucil in Patients With Previously Untreated Chronic Lymphocytic Leukemia. Completed NCT00738374 Phase 2 rituximab [MabThera/Rituxan];chlorambucil
15 A Study to Assess the Efficacy of Rituximab (MabThera) in First Line Treatment of Chronic Lymphocytic Leukemia (CLL) Completed NCT00545714 Phase 2 Cyclophosphamide;Fludarabine;Rituximab
16 Ofatumumab With Fludarabine and Cyclophosphamide in B-CLL Patients Completed NCT00410163 Phase 2 Ofatumumab 500mg;Ofatumumab 1000mg;Fludarabine;Cyclophosphamide
17 Ofatumumab and High-dose Methylprednisolone in Patients With Chronic Lymphocytic Leukemia (CLL) Completed NCT01191190 Phase 2 Ofatumumab/HDMP
18 Alemtuzumab, Fludarabine Phosphate, and Total-Body Irradiation Followed by Cyclosporine and Mycophenolate Mofetil in Treating Patients Who Are Undergoing Donor Stem Cell Transplant for Hematologic Cancer Completed NCT00118352 Phase 2 fludarabine phosphate;cyclosporine;mycophenolate mofetil
19 CRC043: A Phase II Study of Venetoclax in Combination With Dose-adjusted EPOCH-R for Patients With Richter's Syndrome Recruiting NCT03054896 Phase 2 Venetoclax
20 Study of Ibrutinib & Obinutuzumab With/Without CHOP for Richter's Transformation or Richter's Syndrome Patients Recruiting NCT03145480 Phase 2 Obinutuzumab;Ibrutinib
21 Study of Blinatumomab in Richter Transformation Recruiting NCT03121534 Phase 2 Blinatumomab;Dexamethasone
22 Study of Pembrolizumab in Combination With Ublituximab and TGR-1202 in Patients With Relapsed-refractory CLL or Richter's Transformation Recruiting NCT02535286 Phase 1, Phase 2 Pembrolizumab;TGR-1202
23 Trial to Determine the Efficacy and Safety of JCAR017 in Adult Subjects With Aggressive B-Cell Non-Hodgkin Lymphoma Recruiting NCT03484702 Phase 2 JCAR017
24 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 Recruiting NCT02332980 Phase 2 Ibrutinib;Idelalisib
25 Entospletinib and Obinutuzumab in Treating Patients With Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Non-Hodgkin Lymphoma Recruiting NCT03010358 Phase 1, Phase 2 Entospletinib
26 Evaluation of the Safety and Efficacy of the Association of Ibrutinib and Daratumumab in Relapsed/Refractory Chronic Lymphocytic Leukemia With p53 Dysfunction Recruiting NCT03734198 Phase 2 Ibrutinib;Daratumumab
27 Ibrutinib, Fludarabine Phosphate, Cyclophosphamide, and Obinutuzumab in Treating Patients With Chronic Lymphocytic Leukemia Recruiting NCT02629809 Phase 2 Cyclophosphamide;Fludarabine Phosphate;Ibrutinib
28 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) Active, not recruiting NCT02576990 Phase 2
29 Obinutuzumab Containing Conditioning Regimen for Patients With Poor Risk CLL or Richter`s Transformation Requiring Allogeneic Stem Cell Transplantation Active, not recruiting NCT03153514 Phase 2
30 A Study of Acalabrutinib and Vistusertib in Subjects With Relapsed/Refractory B-cell Malignancies Active, not recruiting NCT03205046 Phase 1, Phase 2 acalabrutinib;vistusertib
31 Phase II MOR00208 in Combination With Lenalidomide for Patients With Relapsed or Refractory CLL, SLL or PLL or Older Patients With Untreated CLL, SLL or PLL Active, not recruiting NCT02005289 Phase 2 lenalidomide
32 A Study to Evaluate Safety, Pharmacokinetics, Pharmacodynamics and Preliminary Efficacy of the Combination of Ibrutinib With Nivolumab in Participants With Hematologic Malignancies Active, not recruiting NCT02329847 Phase 1, Phase 2 Ibrutinib;Nivolumab
33 Donor Stem Cell Transplant in Treating Patients With High-Risk Chronic Lymphocytic Leukemia or Small Lymphocytic Lymphoma Active, not recruiting NCT01027000 Phase 2 busulfan;cyclophosphamide;fludarabine phosphate;methotrexate;sirolimus;tacrolimus
34 Ibrutinib Versus Ibrutinib + Rituximab (i vs iR) in Patients With Relapsed Chronic Lymphocytic Leukemia (CLL) Active, not recruiting NCT02007044 Phase 2 Ibrutinib;Rituximab
35 ACP-196 (Acalabrutinib) in Combination With Pembrolizumab, for Treatment of Hematologic Malignancies Active, not recruiting NCT02362035 Phase 1, Phase 2 Acalabrutinib;Pembrolizumab
36 Pentostatin and Lymphocyte Infusion in Preventing Graft Rejection in Patients Who Have Undergone Donor Stem Cell Transplant Active, not recruiting NCT00096161 Phase 2 Cyclosporine;Mycophenolate Mofetil;Pentostatin
37 Trial of Ibrutinib Plus Venetoclax Plus Obinutuzumab in Patients With CLL Active, not recruiting NCT02758665 Phase 2 ibrutinib;obinutuzumab;venetoclax
38 Fludarabine Phosphate, Cyclophosphamide, Total-Body Irradiation, and Donor Bone Marrow Transplant Followed by Donor Natural Killer Cell Therapy, Mycophenolate Mofetil, and Tacrolimus in Treating Patients With Hematologic Cancer Active, not recruiting NCT00789776 Phase 1, Phase 2 Cyclophosphamide;Fludarabine Phosphate;Mycophenolate Mofetil;Tacrolimus
39 Donor Lymphocyte Infusion in Treating Patients With Persistent, Relapsed, or Progressing Cancer After Donor Hematopoietic Cell Transplant Active, not recruiting NCT00068718 Phase 1, Phase 2
40 Tacrolimus and Mycophenolate Mofetil in Preventing Graft-Versus-Host Disease in Patients Who Have Undergone Total-Body Irradiation With or Without Fludarabine Phosphate Followed by Donor Peripheral Blood Stem Cell Transplant for Hematologic Cancer Active, not recruiting NCT00089011 Phase 2 fludarabine phosphate;mycophenolate mofetil;tacrolimus
41 Ibrutinib and PD-1 Blockade in High Risk Lymphocytic Leukemia Not yet recruiting NCT03514017 Phase 2 Pembrolizumab;Ibrutinib
42 Selinexor in Initial or Relapsed/Refractory Richter's Transformation Terminated NCT02138786 Phase 2 selinexor
43 PNT2258 for Treatment of Patients With Richter's Transformation (Brighton) Terminated NCT02378038 Phase 2 PNT2258
44 5-Azacytidine (Azacytidine; Vidaza) in Chronic Lymphocytic Leukemia Terminated NCT00413478 Phase 2 5-Azacytidine
45 Clofarabine, Gemcitabine, and Busulfan Followed by Donor Stem Cell Transplantation in Treating Participants With Chronic Lymphocytic Leukemia (CLL) Terminated NCT01629511 Phase 1, Phase 2 Busulfan;Clofarabine;Gemcitabine;Methotrexate;Tacrolimus
46 Study of Lenalidomide in Previously Untreated, Symptomatic Chronic Lymphocytic Leukemia (CLL) Terminated NCT00751296 Phase 2 Lenalidomide
47 Sotrastaurin Acetate in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia, Small Lymphocytic Leukemia, Prolymphocytic Leukemia, or Richter's Transformation Withdrawn NCT02285244 Phase 2 sotrastaurin acetate
48 Phase II Study of Fludarabine, Cytarabine (ARA-C) and Erwinase IV in Patients With Relapsed or Refractory Hematologic Malignancies Withdrawn NCT02718755 Phase 2 Fludarabine;Cytarabine;Erwinase
49 Lenalidomide After Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancers Completed NCT01254578 Phase 1 Lenalidomide
50 Obinutuzumab, High Dose Methylprednisolone (HDMP), and Lenalidomide for the Treatment of Patients With Richter's Syndrome Recruiting NCT03113695 Phase 1 Obinutuzumab;lenalidomide;HDMP

Search NIH Clinical Center for Richter's Syndrome

Genetic Tests for Richter's Syndrome

Anatomical Context for Richter's Syndrome

MalaCards organs/tissues related to Richter's Syndrome:

41
B Cells, Lymph Node, Liver, Spleen, Myeloid, T Cells, Colon

Publications for Richter's Syndrome

Articles related to Richter's Syndrome:

(show top 50) (show all 224)
# Title Authors Year
1
Isolated Richter's syndrome of the brain: diagnosis in the eye of the beholder. ( 29627880 )
2018
2
Richter syndrome: A rare complication of chronic lymphocytic leukemia or small lymphocytic lymphoma. ( 30156387 )
2018
3
Exosome-carried microRNA-based signature as a cellular trigger for the evolution of chronic lymphocytic leukemia into Richter syndrome. ( 30238808 )
2018
4
miR-125a and miR-34a expression predicts Richter syndrome in chronic lymphocytic leukemia patients. ( 30242085 )
2018
5
Clinical, pathological, and biological characterization of Richter syndrome developing after ibrutinib treatment for relapsed chronic lymphocytic leukemia. ( 29484684 )
2018
6
Richter's syndrome of the central nervous system diagnosed concurrently with chronic lymphocytic leukaemia: A case report and literature review. ( 30313065 )
2018
7
Extranodal Richter's syndrome of the urinary bladder. ( 30337788 )
2018
8
miRNAs involvement in the pathogenesis of Richter's syndrome. ( 30409799 )
2018
9
Cutaneous Richter Syndrome Mimicking Primary Cutaneous CD4-Positive Small/Medium T-cell Lymphoma: Case Report and Review of the Literature. ( 28937430 )
2018
10
Allogeneic Hematopoietic Cell Transplantation for Richter Syndrome: A Single-Center Experience. ( 29126867 )
2018
11
A single-institution retrospective cohort study of first-line R-EPOCH chemoimmunotherapy for Richter syndrome demonstrating complex chronic lymphocytic leukaemia karyotype as an adverse prognostic factor. ( 29193006 )
2018
12
Biology and treatment of Richter syndrome. ( 29692342 )
2018
13
Novel Richter Syndrome Xenograft Models to Study Genetic Architecture, Biology, and Therapy Responses. ( 29735551 )
2018
14
Rapid complete response to blinatumomab as a successful bridge to allogeneic stem cell transplantation in a case of refractory Richter syndrome. ( 29745744 )
2018
15
BCR kinase inhibitors, idelalisib and ibrutinib, are active and effective in Richter syndrome. ( 29974955 )
2018
16
Use of PD-1 (PDCD1) inhibitors for the treatment of Richter syndrome: experience at a single academic centre. ( 30028000 )
2018
17
Richter Syndrome Presenting With Colon Localization. ( 30516680 )
2018
18
Peripheral T-Cell Lymphoma of the Submandibular Salivary Gland as an Unusual Manifestation of Richter's Syndrome: A Case Report and Literature Review. ( 29410924 )
2017
19
Treatment of Richter's Syndrome. ( 29159711 )
2017
20
Richter's Syndrome with Hypercalcemia Induced by Tumor-Associated Production of Parathyroid Hormone-Related Peptide. ( 28203174 )
2017
21
Intravascular large B-cell lymphoma presenting as Richter's syndrome with cerebral involvement in a patient with chronic lymphocytic leukemia. ( 28878900 )
2017
22
Ibrutinib-induced rapid response in chemotherapy-refractory Richter's syndrome. ( 28681549 )
2017
23
Targeting metabolism and survival in chronic lymphocytic leukemia and Richter syndrome cells by a novel NF-κB inhibitor. ( 28860341 )
2017
24
Second lung malignancy and Richter syndrome in chronic lymphocytic leukemia: case report and literature review. ( 28975027 )
2017
25
Classical Hodgkin lymphoma arising in a patient with chronic lymphocytic leukemia (Richter syndrome). ( 29122775 )
2017
26
Factors predicting survival in chronic lymphocytic leukemia patients developing Richter syndrome transformation into Hodgkin lymphoma. ( 28295527 )
2017
27
Biological and molecular characterization of a rare case of cutaneous Richter syndrome. ( 27400669 )
2017
28
Isolated Cardiac Richter Syndrome: a Case Report. ( 27696201 )
2017
29
Richter Syndrome With Extensive Isolated Pleural Extranodal Involvement: The Importance of PET/CT Imaging. ( 28438345 )
2017
30
An update for Richter syndrome - new directions and developments. ( 28439883 )
2017
31
Richter syndrome presenting as Sister Mary Joseph umbilical nodule. ( 28699653 )
2017
32
"Soccer ball" cells to "donut" cells: an unusual case of Richter syndrome. ( 29170196 )
2017
33
Richter's syndrome: Novel and promising therapeutic alternatives. ( 27742070 )
2016
34
EBV-positive Richter's syndrome with laboratory features of Burkitt's lymphoma, in Ibrutinib-treated chronic lymphocytic leukemia. ( 27852142 )
2016
35
Diagnostic dilemmas of high-grade transformation (Richter's syndrome) of chronic lymphocytic leukaemia: results of the phase II National Cancer Research Institute CHOP-OR clinical trial specialist haemato-pathology central review. ( 27345622 )
2016
36
Bilateral acute orbital compartment syndrome secondary to Richter syndrome: the 'tulip' sign. ( 27061968 )
2016
37
Richter Syndrome in Chronic Lymphocytic Leukemia. ( 26830086 )
2016
38
Richter syndrome: pathogenesis and management. ( 27040710 )
2016
39
Synchronous biphenotypic Richter syndrome with Epstein-Barr virus-positive nodal classical Hodgkin lymphoma and bone marrow diffuse large B-cell lymphoma. ( 27079556 )
2016
40
Hematopoietic Cell Transplantation for Richter Syndrome. ( 27375122 )
2016
41
NCRI phase II study of CHOP in combination with ofatumumab in induction and maintenance in newly diagnosed Richter syndrome. ( 27378086 )
2016
42
An 18F-FDG-PET maximum standardized uptake value &amp;gt;10 represents a novel valid marker for discerning Richter's Syndrome. ( 26402256 )
2015
43
Chronic lymphocytic leukemia transformation into high-grade lymphoma: a description of Richter's syndrome in eight dogs. ( 26463596 )
2015
44
Single arm NCRI phase II study of CHOP in combination with Ofatumumab in induction and maintenance for patients with newly diagnosed Richter's syndrome. ( 25775024 )
2015
45
Ibrutinib has some activity in Richter's syndrome. ( 25635530 )
2015
46
Cytogenetic and flow cytometry evaluation of Richter syndrome reveals MYC, CDKN2A, IGH alterations with loss of CD52, CD62L and increase of CD71 antigen expression as the most frequent recurrent abnormalities. ( 25511139 )
2015
47
The efficacy of ibrutinib in the treatment of Richter syndrome. ( 25745187 )
2015
48
BRAF(V600E) mutations are found in Richter syndrome and may allow targeted therapy in a subset of patients. ( 25758903 )
2015
49
Richter syndrome: an aggressive transformation. ( 25806888 )
2015
50
Periorbital Richter Syndrome. ( 26171102 )
2015

Variations for Richter's Syndrome

Cosmic variations for Richter's Syndrome:

9 (show top 50) (show all 552)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM6940532 YES1 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.1474C>T p.P492S 18:724582-724582 2
2 COSM96797 XPO1 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.1711G>A p.E571K 2:61492337-61492337 2
3 COSM6987331 TSC2 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.5197A>G p.T1733A 16:2088263-2088263 2
4 COSM6987097 TSC2 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.5137C>T p.R1713C 16:2088116-2088116 2
5 COSM10834 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.711G>A p.M237I 17:7674252-7674252 2
6 COSM10659 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.817C>T p.R273C 17:7673803-7673803 2
7 COSM10648 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.524G>A p.R175H 17:7675088-7675088 2
8 COSM44057 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.517G>C p.V173L 17:7675095-7675095 2
9 COSM43842 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.770T>C p.L257P 17:7674193-7674193 2
10 COSM10722 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.853G>A p.E285K 17:7673767-7673767 2
11 COSM44327 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.518T>C p.V173A 17:7675094-7675094 2
12 COSM43947 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.614A>G p.Y205C 17:7674917-7674917 2
13 COSM10758 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.659A>G p.Y220C 17:7674872-7674872 2
14 COSM10662 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.743G>A p.R248Q 17:7674220-7674220 2
15 COSM43936 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.857A>T p.E286V 17:7673763-7673763 2
16 COSM6932 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.733G>A p.G245S 17:7674230-7674230 2
17 COSM43970 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.380C>A p.S127Y 17:7675232-7675232 2
18 COSM45416 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.524G>C p.R175P 17:7675088-7675088 2
19 COSM10687 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.527G>A p.C176Y 17:7675085-7675085 2
20 COSM10690 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.473G>A p.R158H 17:7675139-7675139 2
21 COSM44948 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.526T>C p.C176R 17:7675086-7675086 2
22 COSM44623 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,Hodgkin lymphoma c.487T>A p.Y163N 17:7675125-7675125 2
23 COSM45627 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.486C>T p.I162I 17:7675126-7675126 2
24 COSM10996 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.511G>T p.E171* 17:7675101-7675101 2
25 COSM10813 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.394A>G p.K132E 17:7675218-7675218 2
26 COSM44338 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.845G>A p.R282Q 17:7673775-7673775 2
27 COSM11196 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.734G>T p.G245V 17:7674229-7674229 2
28 COSM44821 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.412G>A p.A138T 17:7675200-7675200 2
29 COSM10645 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.527G>T p.C176F 17:7675085-7675085 2
30 COSM43899 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.434T>C p.L145P 17:7675178-7675178 2
31 COSM11287 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.839G>T p.R280I 17:7673781-7673781 2
32 COSM44171 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.840A>T p.R280S 17:7673780-7673780 2
33 COSM43850 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.659A>C p.Y220S 17:7674872-7674872 2
34 COSM43559 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.517G>T p.V173L 17:7675095-7675095 2
35 COSM10704 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.844C>T p.R282W 17:7673776-7673776 2
36 COSM43896 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.818G>C p.R273P 17:7673802-7673802 2
37 COSM10647 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.404G>T p.C135F 17:7675208-7675208 2
38 COSM43903 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.470T>G p.V157G 17:7675142-7675142 2
39 COSM10656 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.742C>T p.R248W 17:7674221-7674221 2
40 COSM44535 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.761T>A p.I254N 17:7674202-7674202 2
41 COSM44002 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.577C>G p.H193D 17:7674954-7674954 2
42 COSM45035 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.761T>G p.I254S 17:7674202-7674202 2
43 COSM44030 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.760A>G p.I254V 17:7674203-7674203 2
44 COSM44571 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,Hodgkin lymphoma c.581T>G p.L194R 17:7674950-7674950 2
45 COSM10705 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.586C>T p.R196* 17:7674945-7674945 2
46 COSM43963 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.396G>C p.K132N 17:7675216-7675216 2
47 COSM43939 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.632C>T p.T211I 17:7674899-7674899 2
48 COSM10777 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.715A>G p.N239D 17:7674248-7674248 2
49 COSM43555 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.736A>G p.M246V 17:7674227-7674227 2
50 COSM11084 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.517G>A p.V173M 17:7675095-7675095 2

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 38)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.75 ATM MLH1 POLB TP53
2
Show member pathways
12.75 ATM MYC NOTCH1 THBS1 TP53
3 12.66 MLH1 MYC NOTCH1 TP53
4
Show member pathways
12.63 ATM IRF4 MYC TP53
5
Show member pathways
12.57 MLH1 MYC NOTCH1 TP53
6
Show member pathways
12.49 ATM MYC POLB TP53
7 12.21 ATM MYC TP53
8 12.16 ATM MYC POLB TP53
9 12.14 MYC THBS1 TP53
10
Show member pathways
12.1 ATM MYC THBS1 TP53
11 12.09 ATM MYC TP53
12
Show member pathways
12.08 ATM THBS1 TP53
13
Show member pathways
12.03 ATM IRF4 MYC TP53
14 12.02 MLH1 MYC TP53
15 11.99 ATM MYC TP53
16 11.97 ATM MYC TP53
17 11.88 MYC THBS1 TP53
18 11.83 ATM MYC NOTCH1 THBS1 TP53
19 11.81 CD38 CD5 NOTCH1
20 11.8 MYC NOTCH1 TP53
21 11.78 ATM MYC TP53
22 11.77 IRF4 MYC TP53
23 11.53 MYC THBS1 TP53
24 11.52 ATM MLH1 TP53
25 11.28 ATM MYC TP53
26 11.22 ATM MLH1 POLB TP53
27 11.21 ATM MYC TP53
28 11.19 MYC TP53
29 11.17 ATM TP53
30 11.17 ATM MLH1
31 11.12 ATM TP53
32 11.11 MLH1 POLB
33 11.09 MYC TP53
34 11.08 ATM MYC TP53
35 10.96 MYC TP53
36 10.87 ATM TP53
37 10.74 ATM MYC NOTCH1 TP53
38
Show member pathways
10.67 ATM MYC TP53

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 nucleoplasm GO:0005654 9.17 ATM IRF4 MLH1 MYC NOTCH1 POLB

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

(show all 24)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 9.93 ATM IRF4 MYC NOTCH1 TP53
2 negative regulation of apoptotic process GO:0043066 9.88 CD38 MYC THBS1 TP53
3 positive regulation of cell proliferation GO:0008284 9.85 CD38 MYC NOTCH1 THBS1
4 positive regulation of gene expression GO:0010628 9.8 ATM MYC NOTCH1 TP53
5 positive regulation of transcription, DNA-templated GO:0045893 9.77 CD38 IRF4 MYC NOTCH1 TP53
6 cytokine-mediated signaling pathway GO:0019221 9.71 IL19 IRF4 MYC TP53
7 response to hypoxia GO:0001666 9.7 ATM CD38 THBS1
8 cellular response to DNA damage stimulus GO:0006974 9.65 ATM MLH1 MYC POLB TP53
9 positive regulation of intrinsic apoptotic signaling pathway GO:2001244 9.62 IL19 TP53
10 response to progesterone GO:0032570 9.61 CD38 THBS1
11 negative regulation of fibroblast proliferation GO:0048147 9.61 MYC TP53
12 reciprocal meiotic recombination GO:0007131 9.6 ATM MLH1
13 sprouting angiogenesis GO:0002040 9.59 NOTCH1 THBS1
14 positive regulation of DNA binding GO:0043388 9.58 IRF4 MYC
15 positive regulation of reactive oxygen species metabolic process GO:2000379 9.57 THBS1 TP53
16 cellular response to gamma radiation GO:0071480 9.56 ATM TP53
17 somatic hypermutation of immunoglobulin genes GO:0016446 9.51 MLH1 POLB
18 intrinsic apoptotic signaling pathway in response to DNA damage GO:0008630 9.5 ATM MLH1 POLB
19 endocardial cushion development GO:0003197 9.48 NOTCH1 THBS1
20 replicative senescence GO:0090399 9.46 ATM TP53
21 positive regulation of response to DNA damage stimulus GO:2001022 9.43 ATM MYC
22 negative regulation of endothelial cell chemotaxis GO:2001027 9.16 NOTCH1 THBS1
23 response to gamma radiation GO:0010332 9.13 MYC POLB TP53
24 cell cycle arrest GO:0007050 9.02 ATM MYC NOTCH1 THBS1 TP53

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

# Name GO ID Score Top Affiliating Genes
1 RNA polymerase II proximal promoter sequence-specific DNA binding GO:0000978 9.26 IRF4 MYC NOTCH1 TP53
2 enzyme binding GO:0019899 8.92 MLH1 NOTCH1 POLB TP53

Sources for Richter's Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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