MCID: RCH001
MIFTS: 49

Richter's Syndrome

Categories: Rare diseases, Cancer diseases, Immune diseases, Blood 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
SNOMED-CT 68 277550009
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 b-cell lymphomas and diffuse large b-cell lymphoma. An important gene associated with Richter's Syndrome is CD5 (CD5 Molecule), and among its related pathways/superpathways are Pathways in cancer and Apoptosis Modulation and Signaling. The drugs Everolimus and Fludarabine have been mentioned in the context of this disorder. Affiliated tissues include b cells, lymph node and liver, and related phenotypes are Decreased TP53 mRNA expression and Reduced mammosphere formation

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 60)
# Related Disease Score Top Affiliating Genes
1 b-cell lymphomas 29.9 CD5 IRF4 MYC TP53
2 diffuse large b-cell lymphoma 29.9 CD5 IRF4 MYC TP53
3 plasmablastic lymphoma 29.7 CD38 MYC NOTCH1
4 leukemia, chronic lymphocytic 29.5 ATM CD38 CD5 NOTCH1 TP53
5 cll/sll 29.5 ATM CD38 CD5 TP53
6 mantle cell lymphoma 29.3 ATM CD5 MYC TP53
7 prolymphocytic leukemia 29.1 ATM CD38 CD5 MYC
8 kotzot-richter syndrome 12.0
9 colon lymphoma 10.7 CD5 IRF4
10 plasma protein metabolism disease 10.6 CD38 CD5
11 splenic diffuse red pulp small b-cell lymphoma 10.6 NOTCH1 TP53
12 tetraploidy 10.6 NOTCH1 TP53
13 invasive bladder transitional cell carcinoma 10.5 NOTCH1 TP53
14 bone squamous cell carcinoma 10.5 NOTCH1 TP53
15 epidural neoplasm 10.5 CD5 TP53
16 adult lymphoma 10.5 CD5 MYC
17 schneiderian carcinoma 10.5 NOTCH1 TP53
18 ectopic thymus 10.5 CD5 FOXN1
19 megaesophagus 10.5 MYC TP53
20 necrotizing sialometaplasia 10.5 CD5 TP53
21 primary effusion lymphoma 10.5 IRF4 MYC
22 synchronous bilateral breast carcinoma 10.4 ATM TP53
23 uterine corpus cancer 10.4 MYC TP53
24 suppressor of tumorigenicity 3 10.4 MYC TP53
25 spinal cord astrocytoma 10.4 NOTCH1 TP53
26 esophagus adenocarcinoma 10.4 MYC TP53
27 leukemia, chronic lymphocytic 2 10.4
28 leukemia, b-cell, chronic 10.4
29 anal squamous cell carcinoma 10.3 MLH1 TP53
30 leukemia 10.3
31 lymphatic system cancer 10.3 CD5 MYC
32 plasma cell neoplasm 10.2 CD38 MYC
33 myxosarcoma 10.2 ATM TP53
34 plasmacytoma 10.2 CD38 IRF4 MYC
35 lymphoma 10.2
36 cerebral primitive neuroectodermal tumor 10.2 MYC TP53
37 mature b-cell neoplasm 10.2 CD38 MYC TP53
38 medullomyoblastoma 10.1 MYC TP53
39 brain glioma 10.1 NOTCH1 TP53
40 pancreas adenocarcinoma 10.1 MYC NOTCH1 TP53
41 ossifying fibroma 10.1 ERCC6 NOTCH1
42 wilms tumor 1 10.0 MYC NOTCH1 TP53
43 fallopian tube carcinoma 9.9 MYC TP53
44 female reproductive organ cancer 9.9 MLH1 MYC TP53
45 familial adenomatous polyposis 9.9 MLH1 MYC TP53
46 gastrointestinal system cancer 9.9 MLH1 MYC TP53
47 hereditary breast ovarian cancer syndrome 9.9 ATM MLH1 TP53
48 b cell prolymphocytic leukemia 9.9 CD38 CD5 MYC TP53
49 li-fraumeni syndrome 9.9 ATM MLH1 TP53
50 adamantinoma of long bones 9.8 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
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased TP53 mRNA expression GR00389-S-5 9.43 TP53 ATM ERCC6
2 Reduced mammosphere formation GR00396-S 9.35 FOXN1 MYC NOTCH1 ATM CD5
3 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-1 9.32 MLH1 TP53 ATM ERCC6
4 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 9.32 MLH1 MYC TP53 ATM CD38 ERCC6
5 Increased TP53 mRNA expression GR00389-S-6 9.16 TP53 ERCC6

MGI Mouse Phenotypes related to Richter's Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.11 CD38 MLH1 MYC ERCC6 FOXN1 ATM
2 hematopoietic system MP:0005397 10.02 CD5 MYC ERCC6 FOXN1 ATM IRF4
3 endocrine/exocrine gland MP:0005379 9.98 CD38 MLH1 MYC FOXN1 ATM TP53
4 homeostasis/metabolism MP:0005376 9.97 CD38 MLH1 MYC ERCC6 FOXN1 ATM
5 immune system MP:0005387 9.91 CD5 MYC ERCC6 FOXN1 ATM IRF4
6 adipose tissue MP:0005375 9.88 IRF4 ERCC6 MYC ATM TP53
7 hearing/vestibular/ear MP:0005377 9.8 ERCC6 MYC FOXN1 TP53 NOTCH1
8 integument MP:0010771 9.76 MLH1 CD5 MYC ERCC6 FOXN1 NOTCH1
9 neoplasm MP:0002006 9.56 MLH1 MYC ERCC6 FOXN1 NOTCH1 ATM
10 pigmentation MP:0001186 8.92 MYC FOXN1 TP53 NOTCH1

Drugs & Therapeutics for Richter's Syndrome

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

(show top 50) (show all 94)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Everolimus Approved Phase 3,Phase 1,Phase 2 159351-69-6 6442177
2
Fludarabine Approved Phase 3,Phase 1,Phase 2 21679-14-1, 75607-67-9 30751
3
Miconazole Approved, Investigational, Vet_approved Phase 3,Phase 2 22916-47-8 4189
4
Mycophenolate mofetil Approved, Investigational Phase 3,Phase 2 128794-94-5 5281078
5
Mycophenolic acid Approved Phase 3,Phase 2 24280-93-1 446541
6
Sirolimus Approved, Investigational Phase 3,Phase 1,Phase 2 53123-88-9 5284616 6436030 46835353
7 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
8 Antibiotics, Antitubercular Phase 3,Phase 2
9 Antifungal Agents Phase 3,Phase 2
10 Anti-Infective Agents Phase 3,Phase 1,Phase 2
11 Antimetabolites Phase 3,Phase 1,Phase 2
12 Antimetabolites, Antineoplastic Phase 3,Phase 1,Phase 2
13 Antirheumatic Agents Phase 3,Phase 1,Phase 2
14 Antitubercular Agents Phase 3,Phase 2
15 Calcineurin Inhibitors Phase 3,Phase 2
16 Cyclosporins Phase 3,Phase 2
17 Dermatologic Agents Phase 3,Phase 2
18 Immunosuppressive Agents Phase 3,Phase 1,Phase 2
19 Vidarabine Phosphate Phase 3,Phase 2
20
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
21
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
22
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
23
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
24
Cytarabine Approved, Investigational Phase 1, Phase 2,Phase 2 147-94-4 6253
25
Oxaliplatin Approved, Investigational Phase 1, Phase 2,Phase 2 61825-94-3 5310940 9887054 43805 6857599
26
rituximab Approved Phase 1, Phase 2,Phase 2 174722-31-7 10201696
27
Vidarabine Approved, Investigational Phase 1, Phase 2,Phase 2 24356-66-9 32326 21704
28
Bevacizumab Approved, Investigational Phase 2 216974-75-3
29
Chlorambucil Approved Phase 2 305-03-3 2708
30
Methylprednisolone Approved, Vet_approved Phase 2,Phase 1 83-43-2 6741
31
Prednisolone Approved, Vet_approved Phase 2,Phase 1 50-24-8 5755
32
alemtuzumab Approved, Investigational Phase 2 216503-57-0
33
Lenalidomide Approved Phase 2,Phase 1 191732-72-6 216326
34
Etoposide Approved Phase 2 33419-42-0 36462
35
Obinutuzumab Approved, Investigational Phase 2,Phase 1 949142-50-1
36
Blinatumomab Approved, Investigational Phase 2 853426-35-4
37
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
38
Pembrolizumab Approved Phase 1, Phase 2,Phase 2 1374853-91-4
39
Idelalisib Approved Phase 2 870281-82-6
40
nivolumab Approved Phase 1, Phase 2 946414-94-4
41
Busulfan Approved, Investigational Phase 2 55-98-1 2478
42
Methotrexate Approved Phase 2 1959-05-2, 59-05-2 126941
43
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492
44
Adenosine Approved, Investigational Phase 2 58-61-7 60961
45
Erythromycin Approved, Investigational, Vet_approved Phase 2 114-07-8 12560
46
Pentostatin Approved, Investigational Phase 2 53910-25-1 40926 439693
47
Azacitidine Approved, Investigational Phase 2 320-67-2 9444
48
Folic Acid Approved, Nutraceutical, Vet_approved Phase 2 59-30-3 6037
49
leucovorin Approved, Nutraceutical Phase 2 58-05-9 143 6006
50
Doxil Approved June 1999 Phase 2 31703

Interventional clinical trials:

(show top 50) (show all 54)
# 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 Study of Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone (CHOP) With Ofatumumab in Patients With Richter's Syndrome Completed NCT01171378 Phase 2 Ofatumumab
4 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
5 Oxaliplatin, Fludarabine, Cytarabine, and Rituximab in Patients With Richter's Transformation and Leukemias Completed NCT00472849 Phase 1, Phase 2 Oxaliplatin;Fludarabine;Cytarabine;Rituximab;Pegfilgrastim
6 Oxaliplatin, Fludarabine, Cytarabine and Rituximab in Richter's Syndrome, Refractory CLL and PLL Completed NCT00452374 Phase 1, Phase 2 Cytarabine;Fludarabine;Oxaliplatin;Rituximab
7 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
8 Efficacy and Safety Study of PCI-32765 Combine With Ofatumumab in CLL Completed NCT01217749 Phase 1, Phase 2 PCI-32765;ofatumumab
9 Fludarabine, Cyclophosphamide, and Rituximab - High Dose Frontline Completed NCT00794820 Phase 2 Fludarabine Phosphate;Cyclophosphamide;Rituximab
10 Bevacizumab in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia Completed NCT00290810 Phase 2
11 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
12 A Study of MabThera (Rituximab) Plus Chlorambucil in Patients With Previously Untreated Chronic Lymphocytic Leukemia. Completed NCT00738374 Phase 2 rituximab [MabThera/Rituxan];chlorambucil
13 Ofatumumab With Fludarabine and Cyclophosphamide in B-CLL Patients Completed NCT00410163 Phase 2 Ofatumumab 500mg;Ofatumumab 1000mg;Fludarabine;Cyclophosphamide
14 Ofatumumab and High-dose Methylprednisolone in Patients With Chronic Lymphocytic Leukemia (CLL) Completed NCT01191190 Phase 2 Ofatumumab/HDMP
15 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
16 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
17 Study of Ibrutinib & Obinutuzumab With/Without CHOP for Richter's Transformation or Richter's Syndrome Patients Recruiting NCT03145480 Phase 2 Obinutuzumab;Ibrutinib
18 Study of Blinatumomab in Richter Transformation Recruiting NCT03121534 Phase 2 Blinatumomab;Dexamethasone
19 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
20 Trial to Determine the Efficacy and Safety of JCAR017 in Adult Subjects With Aggressive B-Cell Non-Hodgkin Lymphoma Recruiting NCT03484702 Phase 2 JCAR017
21 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
22 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
23 Trial of Ibrutinib Plus Venetoclax Plus Obinutuzumab in Patients With CLL Recruiting NCT02758665 Phase 2 ibrutinib;obinutuzumab;venetoclax
24 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
25 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
26 ACP-196 (Acalabrutinib), a Novel Bruton Tyrosine Kinase (Btk) Inhibitor, for Treatment of Chronic Lymphocytic Leukemia Active, not recruiting NCT02029443 Phase 1, Phase 2 acalabrutinib
27 A Study of Acalabrutinib and Vistusertib in Subjects With Relapsed/Refractory B-cell Malignancies Active, not recruiting NCT03205046 Phase 1, Phase 2 acalabrutinib;vistusertib
28 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
29 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
30 Ibrutinib Versus Ibrutinib + Rituximab (i vs iR) in Patients With Relapsed Chronic Lymphocytic Leukemia (CLL) Active, not recruiting NCT02007044 Phase 2 Ibrutinib;Rituximab
31 ACP-196 (Acalabrutinib) in Combination With Pembrolizumab, for Treatment of Hematologic Malignancies Active, not recruiting NCT02362035 Phase 1, Phase 2 Acalabrutinib;Pembrolizumab
32 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
33 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
34 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
35 Ibrutinib and PD-1 Blockade in High Risk Lymphocytic Leukemia Not yet recruiting NCT03514017 Phase 2 Pembrolizumab;Ibrutinib
36 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 Suspended NCT02005289 Phase 2 lenalidomide
37 Selinexor in Initial or Relapsed/Refractory Richter's Transformation Terminated NCT02138786 Phase 2 selinexor
38 PNT2258 for Treatment of Patients With Richter's Transformation (Brighton) Terminated NCT02378038 Phase 2 PNT2258
39 5-Azacytidine (Azacytidine; Vidaza) in Chronic Lymphocytic Leukemia Terminated NCT00413478 Phase 2 5-Azacytidine
40 Study of Lenalidomide in Previously Untreated, Symptomatic Chronic Lymphocytic Leukemia (CLL) Terminated NCT00751296 Phase 2 Lenalidomide
41 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
42 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
43 Lenalidomide After Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancers Completed NCT01254578 Phase 1 Lenalidomide
44 Obinutuzumab, High Dose Methylprednisolone (HDMP), and Lenalidomide for the Treatment of Patients With Richter's Syndrome Recruiting NCT03113695 Phase 1 Obinutuzumab;lenalidomide;HDMP
45 Study to Assess Safety, Tolerability, Pharmacokinetics and Antitumor Activity of AZD4573 in Relapsed/Refractory Haematological Malignancies Recruiting NCT03263637 Phase 1 AZD4573
46 Study of AZD5991 in Relapsed or Refractory Haematologic Malignancies. Recruiting NCT03218683 Phase 1 AZD5991;AZD5991;AZD5991;AZD5991;AZD5991
47 Pevonedistat and Ibrutinib in Treating Participants With Relapsed or Refractory Chronic Lymphocytic Leukemia or Non-Hodgkin Lymphoma Recruiting NCT03479268 Phase 1 Ibrutinib;Pevonedistat
48 Study to Evaluate Safety and Tolerability of XmAb13676 in Patients With CD20-expressing Hematologic Malignancies Recruiting NCT02924402 Phase 1
49 Phase 1 Study Evaluating ZEN003365 in Relapsed/Refractory Lymphoproliferative Malignancies or Relapsed/Refractory AML Withdrawn NCT02238522 Phase 1 ZEN003365
50 Prospective Observational Study on the Management of Patients With Relapsed or Refractory Follicular Lymphoma (OLYMPE) Completed NCT02316613 Rituximab

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, Myeloid, Spleen, Bone, Bone Marrow

Publications for Richter's Syndrome

Articles related to Richter's Syndrome:

(show top 50) (show all 142)
# Title Authors Year
1
Isolated Richter's syndrome of the brain: diagnosis in the eye of the beholder. ( 29627880 )
2018
2
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
3
Treatment of Richter's Syndrome. ( 29159711 )
2017
4
Richter's Syndrome with Hypercalcemia Induced by Tumor-Associated Production of Parathyroid Hormone-Related Peptide. ( 28203174 )
2017
5
Intravascular large B-cell lymphoma presenting as Richter's syndrome with cerebral involvement in a patient with chronic lymphocytic leukemia. ( 28878900 )
2017
6
Ibrutinib-induced rapid response in chemotherapy-refractory Richter's syndrome. ( 28681549 )
2017
7
Richter's syndrome: Novel and promising therapeutic alternatives. ( 27742070 )
2016
8
EBV-positive Richter's syndrome with laboratory features of Burkitt's lymphoma, in Ibrutinib-treated chronic lymphocytic leukemia. ( 27852142 )
2016
9
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
10
An 18F-FDG-PET maximum standardized uptake value >10 represents a novel valid marker for discerning Richter's Syndrome. ( 26402256 )
2015
11
Chronic lymphocytic leukemia transformation into high-grade lymphoma: a description of Richter's syndrome in eight dogs. ( 26463596 )
2015
12
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
13
Ibrutinib has some activity in Richter's syndrome. ( 25635530 )
2015
14
Cutaneous Richter's syndrome: A better place to transform? ( 24935194 )
2014
15
Hemophagocytic Lymphohistiocytosis With Cerebral Involvement in Richter's Syndrome. ( 25534389 )
2014
16
Platinum and high-dose cytarabine-based regimens are efficient in ultra high/high-risk chronic lymphocytic leukemia and Richter's syndrome: results of a French retrospective multicenter study. ( 25359318 )
2014
17
Testicular swelling: a rare manifestation of chronic lymphocytic leukemia presenting with Richter's syndrome. ( 24739853 )
2014
18
Richter's syndrome: Utility of A^a8,F-FDG PET/CT. ( 23044071 )
2013
19
Richter's syndrome with aberrant CD2 and CD4 expression. ( 22649134 )
2012
20
Autologous and allogeneic stem-cell transplantation for transformed chronic lymphocytic leukemia (Richter's syndrome): A retrospective analysis from the chronic lymphocytic leukemia subcommittee of the chronic leukemia working party and lymphoma working party of the European Group for Blood and Marrow Transplantation. ( 22547610 )
2012
21
Richter's syndrome in colon: case report. ( 21057893 )
2012
22
Richter's syndrome: CLL taking a turn for the worse. ( 23413593 )
2012
23
Richter's syndrome of the brain and the spinal cord. ( 20587487 )
2011
24
MDR-1 gene polymorphisms G2677T and C3435T in a case of Hodgkin's variant of Richter's syndrome. ( 22866091 )
2011
25
Genomic profiling of Richter's syndrome: recurrent lesions and differences with de novo diffuse large B-cell lymphomas. ( 20014148 )
2010
26
Cutaneous Richter's syndrome, prognosis, and clinical, histological and immunohistological patterns: report of four cases and review of the literature. ( 20145381 )
2010
27
Hodgkin's lymphoma variant of Richter's syndrome. ( 20437646 )
2010
28
Haemophagocytic syndrome and Hodgkin's disease variant of Richter's syndrome after fludarabine for CLL. ( 20331737 )
2010
29
Intrathecal liposomal cytarabine for treatment of leptomeningeal involvement in transformed (Richter's syndrome) and non-transformed B-cell chronic lymphocytic leukaemia in Spain: a report of seven cases. ( 20487439 )
2010
30
Telomere length is an independent predictor of survival, treatment requirement and Richter's syndrome transformation in chronic lymphocytic leukemia. ( 19340005 )
2009
31
Richter's syndrome: a case report. ( 19204436 )
2009
32
Richter's syndrome developing in a patient with adult onset Still's disease. ( 18607590 )
2009
33
[Clonality analysis and mutation status of IgVH genes in classic Richter's syndrome]. ( 19031714 )
2008
34
Phase I-II study of oxaliplatin, fludarabine, cytarabine, and rituximab combination therapy in patients with Richter's syndrome or fludarabine-refractory chronic lymphocytic leukemia. ( 18182662 )
2008
35
Recent advances in the diagnosis and therapy of Richter's syndrome. ( 17673808 )
2007
36
Primary effusion lymphoma presenting as Richter's syndrome. ( 17573910 )
2007
37
EBV negative Richter's syndrome from a coexistent clone after salvage treatment with alemtuzumab in a CLL patient. ( 16838338 )
2006
38
Chronic lymphocytic leukemia with osteolytic Richter's syndrome mimicking myeloma bone disease shows no over-expression of DKK1. ( 17065022 )
2006
39
Clinical outcomes and prognostic factors in patients with Richter's syndrome treated with chemotherapy or chemoimmunotherapy with or without stem-cell transplantation. ( 16710033 )
2006
40
Hodgkin's disease variant of Richter's syndrome clonally related to chronic lymphocytic leukemia arises in ZAP-70 negative mutated CLL. ( 16223567 )
2006
41
Cat scratch disease mimicking Richter's Syndrome in a patient with chronic lymphocytic leukemia. ( 15621836 )
2005
42
Unusual sites of malignancies: CASE 2. Central neurogenic hyperventilation as a complication of Richter's syndrome. ( 15774798 )
2005
43
Richter's syndrome--the downside of fludarabine? ( 16111529 )
2005
44
Richter's syndrome: biology and therapy. ( 16053658 )
2005
45
[Isolated Richter's syndrome in central nervous system: case report]. ( 16059612 )
2005
46
Hodgkin's disease variant of Richter's syndrome in chronic lymphocytic leukaemia patients previously treated with fludarabine. ( 15813847 )
2005
47
Richter's syndrome in the brain first manifested as an ischaemic stroke. ( 15360010 )
2004
48
Distinct clonal origin in two cases of Hodgkin's lymphoma variant of Richter's syndrome associated With EBV infection. ( 15105659 )
2004
49
Richter's syndrome presenting as a nasal lymphoma. ( 15223655 )
2004
50
Secondary follicular lymphoma of the bone, transformed into large cell lymphoma, in a patient with chronic lymphocytic leukaemia: an uncommon manifestation of Richter's syndrome. ( 14962241 )
2004

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 COSM6987097 TSC2 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.5137C>T p.R1713C 16:2088116-2088116 2
4 COSM6987331 TSC2 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.5197A>G p.T1733A 16:2088263-2088263 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 COSM43967 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.751A>T p.I251F 17:7674212-7674212 2
8 COSM10656 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.742C>T p.R248W 17:7674221-7674221 2
9 COSM43684 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.720T>G p.S240R 17:7674243-7674243 2
10 COSM10648 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.524G>A p.R175H 17:7675088-7675088 2
11 COSM44057 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.517G>C p.V173L 17:7675095-7675095 2
12 COSM10742 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.578A>G p.H193R 17:7674953-7674953 2
13 COSM10722 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.853G>A p.E285K 17:7673767-7673767 2
14 COSM44327 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.518T>C p.V173A 17:7675094-7675094 2
15 COSM43947 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.614A>G p.Y205C 17:7674917-7674917 2
16 COSM10758 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.659A>G p.Y220C 17:7674872-7674872 2
17 COSM10662 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.743G>A p.R248Q 17:7674220-7674220 2
18 COSM43936 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.857A>T p.E286V 17:7673763-7673763 2
19 COSM6932 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.733G>A p.G245S 17:7674230-7674230 2
20 COSM43970 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.380C>A p.S127Y 17:7675232-7675232 2
21 COSM45416 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.524G>C p.R175P 17:7675088-7675088 2
22 COSM10687 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.527G>A p.C176Y 17:7675085-7675085 2
23 COSM10690 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.473G>A p.R158H 17:7675139-7675139 2
24 COSM44948 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.526T>C p.C176R 17:7675086-7675086 2
25 COSM44623 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,Hodgkin lymphoma c.487T>A p.Y163N 17:7675125-7675125 2
26 COSM45627 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.486C>T p.I162I 17:7675126-7675126 2
27 COSM10996 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.511G>T p.E171* 17:7675101-7675101 2
28 COSM10813 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.394A>G p.K132E 17:7675218-7675218 2
29 COSM44338 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.845G>A p.R282Q 17:7673775-7673775 2
30 COSM11196 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.734G>T p.G245V 17:7674229-7674229 2
31 COSM44821 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.412G>A p.A138T 17:7675200-7675200 2
32 COSM10645 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.527G>T p.C176F 17:7675085-7675085 2
33 COSM43899 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.434T>C p.L145P 17:7675178-7675178 2
34 COSM11287 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.839G>T p.R280I 17:7673781-7673781 2
35 COSM44171 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.840A>T p.R280S 17:7673780-7673780 2
36 COSM43850 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.659A>C p.Y220S 17:7674872-7674872 2
37 COSM43559 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.517G>T p.V173L 17:7675095-7675095 2
38 COSM10704 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.844C>T p.R282W 17:7673776-7673776 2
39 COSM43896 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.818G>C p.R273P 17:7673802-7673802 2
40 COSM10647 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.404G>T p.C135F 17:7675208-7675208 2
41 COSM43903 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.470T>G p.V157G 17:7675142-7675142 2
42 COSM44535 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.761T>A p.I254N 17:7674202-7674202 2
43 COSM44002 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.577C>G p.H193D 17:7674954-7674954 2
44 COSM45035 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.761T>G p.I254S 17:7674202-7674202 2
45 COSM44030 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.760A>G p.I254V 17:7674203-7674203 2
46 COSM44571 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,Hodgkin lymphoma c.581T>G p.L194R 17:7674950-7674950 2
47 COSM10705 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.586C>T p.R196* 17:7674945-7674945 2
48 COSM43963 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.396G>C p.K132N 17:7675216-7675216 2
49 COSM43939 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.632C>T p.T211I 17:7674899-7674899 2
50 COSM10777 TP53 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.715A>G p.N239D 17:7674248-7674248 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 29)
# Super pathways Score Top Affiliating Genes
1 12.61 MLH1 MYC NOTCH1 TP53
2
Show member pathways
12.59 ATM IRF4 MYC TP53
3
Show member pathways
12.5 MLH1 MYC NOTCH1 TP53
4
Show member pathways
12.42 ATM ERCC6 MYC TP53
5 12.18 ATM MYC TP53
6 12.18 ATM MYC NOTCH1 TP53
7 12.1 CD38 MYC TP53
8 12.04 ATM MYC TP53
9 11.97 MLH1 MYC TP53
10 11.94 ATM MYC TP53
11 11.91 ATM MYC TP53
12 11.74 CD38 CD5 NOTCH1
13 11.72 MYC NOTCH1 TP53
14 11.69 ATM MYC TP53
15 11.67 IRF4 MYC TP53
16 11.41 ATM MLH1 TP53
17 11.2 ATM MYC TP53
18 11.16 MYC TP53
19 11.14 ATM TP53
20 11.13 ATM MLH1
21 11.1 ATM MYC TP53
22 11.08 ATM TP53
23 11.07 ERCC6 MLH1
24 11.05 MYC TP53
25 10.98 ATM MYC TP53
26 10.91 MYC TP53
27 10.8 ATM TP53
28 10.74 ATM MYC NOTCH1 TP53
29
Show member pathways
10.47 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 ERCC6 IRF4 MLH1 MYC NOTCH1

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

(show all 18)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 9.92 FOXN1 IRF4 MYC NOTCH1 TP53
2 cell proliferation GO:0008283 9.8 CD5 FOXN1 MYC TP53
3 transcription by RNA polymerase II GO:0006366 9.77 ERCC6 FOXN1 IRF4 MYC TP53
4 positive regulation of transcription, DNA-templated GO:0045893 9.72 CD38 IRF4 MYC NOTCH1 TP53
5 reciprocal meiotic recombination GO:0007131 9.58 ATM MLH1
6 negative regulation of fibroblast proliferation GO:0048147 9.57 MYC TP53
7 cellular response to gamma radiation GO:0071480 9.56 ATM TP53
8 male meiotic nuclear division GO:0007140 9.55 ATM MLH1
9 cellular response to DNA damage stimulus GO:0006974 9.55 ATM ERCC6 MLH1 MYC TP53
10 positive regulation of DNA binding GO:0043388 9.54 IRF4 MYC
11 response to X-ray GO:0010165 9.51 ERCC6 TP53
12 replicative senescence GO:0090399 9.48 ATM TP53
13 determination of adult lifespan GO:0008340 9.46 ATM TP53
14 positive regulation of response to DNA damage stimulus GO:2001022 9.43 ATM MYC
15 intrinsic apoptotic signaling pathway in response to DNA damage GO:0008630 9.43 ATM ERCC6 MLH1
16 meiotic telomere clustering GO:0045141 9.4 ATM MLH1
17 cell cycle arrest GO:0007050 9.26 ATM MYC NOTCH1 TP53
18 response to gamma radiation GO:0010332 8.8 ERCC6 MYC TP53

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

# Name GO ID Score Top Affiliating Genes
1 DNA binding transcription factor activity GO:0003700 9.65 FOXN1 IRF4 MYC NOTCH1 TP53
2 protein-containing complex binding GO:0044877 9.33 ATM ERCC6 MYC
3 chromatin binding GO:0003682 9.26 ERCC6 MLH1 NOTCH1 TP53
4 protein N-terminus binding GO:0047485 8.8 ATM ERCC6 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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