MCID: RCH001
MIFTS: 45

Richter's Syndrome malady

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

Aliases & Classifications for Richter's Syndrome

Aliases & Descriptions for Richter's Syndrome:

Name: Richter's Syndrome 12 14 69
Richter Syndrome 12 50
Richter Transformation 50

Classifications:



External Ids:

Disease Ontology 12 DOID:1703
ICD10 33 C91.1
NCIt 47 C35424
SNOMED-CT 64 277550009
UMLS 69 C0349631

Summaries for Richter's Syndrome

NIH Rare Diseases : 50 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). last updated: 1/3/2014

MalaCards based summary : Richter's Syndrome, also known as richter syndrome, is related to kotzot-richter syndrome and leukemia. An important gene associated with Richter's Syndrome is CD5 (CD5 Molecule), and among its related pathways/superpathways are Apoptosis Modulation and Signaling and MicroRNAs in cancer. The drugs Dopamine and Aripiprazole have been mentioned in the context of this disorder. Affiliated tissues include lymph node, liver and spleen, and related phenotypes are Increased shRNA abundance (Z-score > 2) and hematopoietic system

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 55)
id Related Disease Score Top Affiliating Genes
1 kotzot-richter syndrome 11.9
2 leukemia 10.3
3 chronic lymphocytic leukemia 10.3
4 congenital sucrase-isomaltase deficiency without sucrose intolerance 10.2 NOTCH1 TP53
5 penile disease 10.2 CD5 IRF4
6 breast epithelioid hemangioma 10.2 NOTCH1 TP53
7 polydactyly cleft lip palate psychomotor retardation 10.2 CD38 NOTCH1
8 brain stem medulloblastoma 10.2 NOTCH1 TP53
9 heterophyiasis 10.1 CRP NOTCH1
10 villoglandular endometrial endometrioid adenocarcinoma 10.1 ATM TP53
11 familial hypopituitarism 10.1 MLH1 TP53
12 endometrial squamous cell carcinoma 10.1 MLH1 TP53
13 prinzmetal's variant angina 10.1 IRF4 MYC
14 lymphoma 10.1
15 theileriasis 10.1 CD38 CD5 MYC
16 spinal cord intramedullary teratoma 10.1 CD5 TP53
17 anterior foramen magnum meningioma 10.1 CD38 MYC TP53
18 gall bladder carcinoma in situ 10.1 MYC MYCN
19 postpartum depression 10.1 MYC TP53
20 pineoblastoma 10.0 MYCN TP53
21 asperger syndrome 10.0 MLH1 MYC TP53
22 b-cell lymphomas 10.0
23 trachea squamous cell carcinoma 10.0 MLH1 MYC TP53
24 ectodermal dysplasia 10.0 CD5 IRF4 MYC TP53
25 barnicoat baraitser syndrome 10.0 CD38 CD5 MYC TP53
26 bladder disease 10.0 CD5 IRF4 MYC TP53
27 bardet-biedl syndrome 10.0 MLH1 MYC TP53
28 proctitis 10.0 MLH1 MYC TP53
29 nevoid hypermelanosis, linear and whorled 10.0 MLH1 MYC TP53
30 nasopharyngeal carcinoma 10.0 ATM MLH1 TP53
31 peripheral osteosarcoma 10.0 MYC MYCN
32 childhood brain germinoma 10.0 MYC MYCN TP53
33 childhood medulloblastoma 10.0 MYC MYCN TP53
34 extragonadal germ cell cancer 10.0 MYC MYCN TP53
35 childhood brain stem glioma 10.0 ATM CD38 CD5 TP53
36 cutis laxa, autosomal recessive, type ib 10.0 CD38 IRF4 MYC TP53
37 postmenopausal atrophic vaginitis 10.0 MYC MYCN TP53
38 diffuse large b-cell lymphoma 10.0
39 cystadenoma 10.0 MYC MYCN TP53
40 thoracic outlet syndrome 10.0 MYC MYCN TP53
41 seminal vesicle chronic gonorrhea 10.0 ATM CD38 CD5 MYC
42 cranioectodermal dysplasia 1 10.0 CD38 CD5 MYC TP53
43 rhabdomyosarcoma, embryonal, 2 9.9 MYCN TP53
44 melanoma metastasis 9.9 ATM CD5 MYC TP53
45 uterine corpus endometrial stromal sarcoma 9.9 MLH1 TP53
46 neuroblastoma 9.8 CD38 MYC MYCN TP53
47 pulmonary fibrosis, idiopathic 9.7 ATM CD38 CD5 IRF4 MYC TP53
48 chronic venous leg ulcers 9.7 ATM CD38 CD5 MYC NOTCH1 TP53
49 follicular lymphoma 9.6
50 hematopoietic stem cell transplantation 9.6

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
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-12 9.7 TP53
2 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.7 CRP MYCN NOTCH1 TP53
3 Increased shRNA abundance (Z-score > 2) GR00366-A-132 9.7 TP53
4 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.7 MYCN NOTCH1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.7 NOTCH1 CRP
6 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.7 NOTCH1 CRP
7 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.7 TP53
8 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.7 MYCN
9 Increased shRNA abundance (Z-score > 2) GR00366-A-19 9.7 TP53
10 Increased shRNA abundance (Z-score > 2) GR00366-A-190 9.7 TP53
11 Increased shRNA abundance (Z-score > 2) GR00366-A-205 9.7 NOTCH1
12 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.7 NOTCH1
13 Increased shRNA abundance (Z-score > 2) GR00366-A-35 9.7 TP53
14 Increased shRNA abundance (Z-score > 2) GR00366-A-39 9.7 TP53
15 Increased shRNA abundance (Z-score > 2) GR00366-A-49 9.7 CRP
16 Increased shRNA abundance (Z-score > 2) GR00366-A-6 9.7 NOTCH1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.7 CRP
18 Increased shRNA abundance (Z-score > 2) GR00366-A-73 9.7 NOTCH1

MGI Mouse Phenotypes related to Richter's Syndrome:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.86 ATM CD38 CD5 IRF4 MYC MYCN
2 immune system MP:0005387 9.76 ATM CD38 CD5 CRP IRF4 MYC
3 integument MP:0010771 9.43 ATM CD5 MLH1 MYC NOTCH1 TP53
4 neoplasm MP:0002006 9.1 NOTCH1 TP53 ATM IRF4 MLH1 MYC

Drugs & Therapeutics for Richter's Syndrome

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

(show top 50) (show all 100)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
2
Aripiprazole Approved, Investigational Phase 3 129722-12-9 60795
3
Abacavir Approved, Investigational Phase 3 136470-78-5 65140 441300
4
Lamivudine Approved, Investigational Phase 3 134678-17-4 60825
5
Lopinavir Approved Phase 3 192725-17-0 92727
6
Zidovudine Approved Phase 3 30516-87-1 35370
7
Lenalidomide Approved Phase 3,Phase 2,Phase 1 191732-72-6 216326
8 Antipsychotic Agents Phase 3
9 Dopamine Agents Phase 3
10 Psychotropic Drugs Phase 3
11 Central Nervous System Depressants Phase 3
12 Tranquilizing Agents Phase 3
13 Anti-HIV Agents Phase 3
14 Anti-Infective Agents Phase 3,Phase 1,Phase 2
15 Antimetabolites Phase 3,Phase 1,Phase 2
16 Anti-Retroviral Agents Phase 3
17 Antiviral Agents Phase 3,Phase 1,Phase 2
18 Lamivudine, zidovudine drug combination Phase 3
19 Nucleic Acid Synthesis Inhibitors Phase 3
20 Reverse Transcriptase Inhibitors Phase 3
21 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
22 Immunosuppressive Agents Phase 3,Phase 1,Phase 2
23 Angiogenesis Inhibitors Phase 3,Phase 2,Phase 1
24 Angiogenesis Modulating Agents Phase 3,Phase 2,Phase 1
25
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
26
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
27
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
28
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
29
Cytarabine Approved, Investigational Phase 1, Phase 2 147-94-4 6253
30
Fludarabine Approved Phase 1, Phase 2 21679-14-1, 75607-67-9 30751
31
Oxaliplatin Approved, Investigational Phase 1, Phase 2 61825-94-3 5310940 9887054 6857599, 9887054 43805
32
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
33
Vidarabine Approved Phase 1, Phase 2 24356-66-9 32326 21704
34
Etoposide Approved Phase 2 33419-42-0 36462
35
Obinutuzumab Approved Phase 2,Phase 1 949142-50-1
36
Pembrolizumab Approved Phase 2,Phase 1 1374853-91-4
37
nivolumab Approved Phase 1, Phase 2 946414-94-4
38
Idelalisib Approved Phase 2 870281-82-6
39
Blinatumomab Approved Phase 2 853426-35-4
40
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
41
Asparaginase Approved Phase 2 9015-68-3
42
Azacitidine Approved, Investigational Phase 2 320-67-2 9444
43
Doxil Approved June 1999 Phase 2 31703
44 Antibodies Phase 2,Phase 1
45 Antibodies, Monoclonal Phase 2,Phase 1
46 Immunoglobulins Phase 2,Phase 1
47 Antimetabolites, Antineoplastic Phase 1, Phase 2
48 Antirheumatic Agents Phase 1, Phase 2
49 Alkylating Agents Phase 2
50 Antibiotics, Antitubercular Phase 2

Interventional clinical trials:

(show all 41)
id Name Status NCT ID Phase
1 Treatment of Predominant Central Sleep Apnoea by Adaptive Servo Ventilation in Patients With Heart Failure Completed NCT00733343 Phase 4
2 Safety and Efficacy of Cariprazine in Schizophrenia Completed NCT01104779 Phase 3
3 Safety and Efficacy of Cariprazine in Patients With Schizophrenia Completed NCT01104766 Phase 3
4 FREE Study: Efficacy and Toxicity of Trizivir Completed NCT00405925 Phase 3
5 Community Mobilization, Mobile Testing, Same-Day Results, and Post-Test Support for HIV in Sub-Saharan Africa and Thailand Completed NCT00203749 Phase 3
6 Elevate CLL R/R: Study of Acalabrutinib (ACP-196) Versus Ibrutinib in Previously Treated Subjects With High Risk Chronic Lymphocytic Leukemia Recruiting NCT02477696 Phase 3
7 A Study of Lenalidomide Versus Placebo in Subjects With Transfusion Dependent Anemia in Lower Risk Myelodysplastic Syndrome (MDS) Without Del 5q Active, not recruiting NCT01029262 Phase 3
8 Study of Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone (CHOP) With Ofatumumab in Patients With Richter's Syndrome Unknown status NCT01171378 Phase 2
9 VNP40101M in Treating Patients With Richter Syndrome or Refractory or Relapsed Chronic Lymphocytic Leukemia or Other Lymphoproliferative Disorders Completed NCT00304005 Phase 1, Phase 2
10 Oxaliplatin, Fludarabine, Cytarabine, and Rituximab in Patients With Richter's Transformation and Leukemias Completed NCT00472849 Phase 1, Phase 2
11 Oxaliplatin, Fludarabine, Cytarabine and Rituximab in Richter's Syndrome, Refractory CLL and PLL Completed NCT00452374 Phase 1, Phase 2
12 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
13 Efficacy and Safety Study of PCI-32765 Combine With Ofatumumab in CLL Completed NCT01217749 Phase 1, Phase 2
14 A Phase II Study of Venetoclax in Combination With Dose-adjusted EPOCH-R for Patients With Richter's Syndrome Recruiting NCT03054896 Phase 2
15 Study of Ibrutinib & Obinutuzumab With/Without CHOP for Richter's Transformation or Richter's Syndrome Patients Recruiting NCT03145480 Phase 2
16 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) Recruiting NCT02576990 Phase 2
17 Obinutuzumab Containing Conditioning Regimen for Patients With Poor Risk CLL or Richter`s Transformation Requiring Allogeneic Stem Cell Transplantation Recruiting NCT03153514 Phase 2
18 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
19 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 Recruiting NCT02005289 Phase 2
20 A Study to Evaluate Safety, Pharmacokinetics, Pharmacodynamics and Preliminary Efficacy of the Combination of Ibrutinib With Nivolumab in Participants With Hematologic Malignancies Recruiting NCT02329847 Phase 1, Phase 2
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
22 Trial of Ibrutinib Plus Venetoclax Plus Obinutuzumab in Patients With CLL Recruiting NCT02758665 Phase 2
23 ACP-196 (Acalabrutinib), a Novel Bruton Tyrosine Kinase (Btk) Inhibitor, for Treatment of Chronic Lymphocytic Leukemia Active, not recruiting NCT02029443 Phase 1, Phase 2
24 ACP-196 (Acalabrutinib) in Combination With Pembrolizumab, for Treatment of Hematologic Malignancies Active, not recruiting NCT02362035 Phase 1, Phase 2
25 Study of Blinatumomab in Richter Transformation Not yet recruiting NCT03121534 Phase 2
26 Entospletinib and Obinutuzumab in Treating Patients With Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Non-Hodgkin Lymphoma Not yet recruiting NCT03010358 Phase 1, Phase 2
27 Phase II Study of Fludarabine, Cytarabine (ARA-C) and Erwinase IV in Patients With Relapsed or Refractory Hematologic Malignancies Not yet recruiting NCT02718755 Phase 2
28 Selinexor in Initial or Relapsed/Refractory Richter's Transformation Terminated NCT02138786 Phase 2
29 PNT2258 for Treatment of Patients With Richter's Transformation (Brighton) Terminated NCT02378038 Phase 2
30 5-Azacytidine (Azacytidine; Vidaza) in Chronic Lymphocytic Leukemia Terminated NCT00413478 Phase 2
31 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
32 Lenalidomide After Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancers Active, not recruiting NCT01254578 Phase 1
33 Obinutuzumab, High Dose Methylprednisolone (HDMP), and Lenalidomide for the Treatment of Patients With Richter's Syndrome Not yet recruiting NCT03113695 Phase 1
34 Phase 1 Study Evaluating ZEN003365 in Relapsed/Refractory Lymphoproliferative Malignancies or Relapsed/Refractory AML Withdrawn NCT02238522 Phase 1
35 Detrol LA vs Estrace Vaginal Cream for the Treatment of Overactive Bladder Symptoms Completed NCT00465894
36 Ekvasis of Atorvastatin (Antorcin®) Treatment in Patients With Acute Cardiovascular Events Completed NCT01770210
37 Registry of the German CLL Study Group Recruiting NCT02863692
38 Identification of Biomarkers That Are Predictive of Early Ibrutinib Treatment Failure in High Risk TP53 Mutated Chronic Lymphocytic Leukemia Recruiting NCT02827617
39 Clinical-biological Characteristics and Outcome of Chronic Lymphocytic Leukemia Under Ibrutinib-named Patient Program Recruiting NCT02582320
40 Prospective Analysis of Septic Associated Encephalopathy Using the Non-invasive Acoustocerebrography-ACG Recruiting NCT03173196
41 The Use of Psychomotor Vigilance Testing in the Assessment of Pediatric Obstructive Sleep Apnea Active, not recruiting NCT01276860

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:

39
Lymph Node, Liver, Spleen, B Cells, Bone, Brain, T Cells

Publications for Richter's Syndrome

Articles related to Richter's Syndrome:

(show top 50) (show all 135)
id Title Authors Year
1
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
2
EBV-positive Richter's syndrome with laboratory features of Burkitt's lymphoma, in Ibrutinib-treated chronic lymphocytic leukemia. ( 27852142 )
2016
3
Richter's syndrome: Novel and promising therapeutic alternatives. ( 27742070 )
2016
4
Ibrutinib has some activity in Richter's syndrome. ( 25635530 )
2015
5
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
6
Chronic lymphocytic leukemia transformation into high-grade lymphoma: a description of Richter's syndrome in eight dogs. ( 26463596 )
2015
7
An 18F-FDG-PET maximum standardized uptake value >10 represents a novel valid marker for discerning Richter's Syndrome. ( 26402256 )
2015
8
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
9
Hemophagocytic Lymphohistiocytosis With Cerebral Involvement in Richter's Syndrome. ( 25534389 )
2014
10
Cutaneous Richter's syndrome: A better place to transform? ( 24935194 )
2014
11
Testicular swelling: a rare manifestation of chronic lymphocytic leukemia presenting with Richter's syndrome. ( 24739853 )
2014
12
Richter's syndrome: Utility of A^a8,F-FDG PET/CT. ( 23044071 )
2013
13
Richter's syndrome: CLL taking a turn for the worse. ( 23413593 )
2012
14
Richter's syndrome with aberrant CD2 and CD4 expression. ( 22649134 )
2012
15
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
16
Richter's syndrome in colon: case report. ( 21057893 )
2012
17
MDR-1 gene polymorphisms G2677T and C3435T in a case of Hodgkin's variant of Richter's syndrome. ( 22866091 )
2011
18
Richter's syndrome of the brain and the spinal cord. ( 20587487 )
2011
19
Genomic profiling of Richter's syndrome: recurrent lesions and differences with de novo diffuse large B-cell lymphomas. ( 20014148 )
2010
20
Haemophagocytic syndrome and Hodgkin's disease variant of Richter's syndrome after fludarabine for CLL. ( 20331737 )
2010
21
Cutaneous Richter's syndrome, prognosis, and clinical, histological and immunohistological patterns: report of four cases and review of the literature. ( 20145381 )
2010
22
Hodgkin's lymphoma variant of Richter's syndrome. ( 20437646 )
2010
23
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
24
Richter's syndrome: a case report. ( 19204436 )
2009
25
Richter's syndrome developing in a patient with adult onset Still's disease. ( 18607590 )
2009
26
Telomere length is an independent predictor of survival, treatment requirement and Richter's syndrome transformation in chronic lymphocytic leukemia. ( 19340005 )
2009
27
[Clonality analysis and mutation status of IgVH genes in classic Richter's syndrome]. ( 19031714 )
2008
28
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
29
Recent advances in the diagnosis and therapy of Richter's syndrome. ( 17673808 )
2007
30
Primary effusion lymphoma presenting as Richter's syndrome. ( 17573910 )
2007
31
Clinical outcomes and prognostic factors in patients with Richter's syndrome treated with chemotherapy or chemoimmunotherapy with or without stem-cell transplantation. ( 16710033 )
2006
32
Chronic lymphocytic leukemia with osteolytic Richter's syndrome mimicking myeloma bone disease shows no over-expression of DKK1. ( 17065022 )
2006
33
EBV negative Richter's syndrome from a coexistent clone after salvage treatment with alemtuzumab in a CLL patient. ( 16838338 )
2006
34
Hodgkin's disease variant of Richter's syndrome clonally related to chronic lymphocytic leukemia arises in ZAP-70 negative mutated CLL. ( 16223567 )
2006
35
Richter's syndrome: biology and therapy. ( 16053658 )
2005
36
Hodgkin's disease variant of Richter's syndrome in chronic lymphocytic leukaemia patients previously treated with fludarabine. ( 15813847 )
2005
37
Unusual sites of malignancies: CASE 2. Central neurogenic hyperventilation as a complication of Richter's syndrome. ( 15774798 )
2005
38
Richter's syndrome--the downside of fludarabine? ( 16111529 )
2005
39
Cat scratch disease mimicking Richter's Syndrome in a patient with chronic lymphocytic leukemia. ( 15621836 )
2005
40
[Isolated Richter's syndrome in central nervous system: case report]. ( 16059612 )
2005
41
Relationship between the mutational status of VH genes and pathogenesis of diffuse large B-cell lymphoma in Richter's syndrome. ( 14671632 )
2004
42
Richter's syndrome in the brain first manifested as an ischaemic stroke. ( 15360010 )
2004
43
Distinct clonal origin in two cases of Hodgkin's lymphoma variant of Richter's syndrome associated With EBV infection. ( 15105659 )
2004
44
Second malignancies and Richter's syndrome in patients with chronic lymphocytic leukemia. ( 15763979 )
2004
45
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
46
Second malignancies and Richter's syndrome in patients with chronic lymphocytic leukaemia treated with cladribine. ( 14746857 )
2004
47
Richter's syndrome presenting as a nasal lymphoma. ( 15223655 )
2004
48
Richter's syndrome: a novel presentation. ( 12868708 )
2003
49
Hodgkin's type of Richter's syndrome in familial chronic lymphocytic leukemia treated with cladribine and cyclophosphamide. ( 12802926 )
2003
50
Variant Richter's syndrome: a rare case of classical Hodgkin's lymphoma developing in a patient with chronic lymphocytic leukemia treated with fludarabine. ( 14959863 )
2003

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 26)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.59 ATM IRF4 MYC TP53
2 12.23 ATM MYC NOTCH1 TP53
3 12.11 CD38 MYC TP53
4 11.97 MLH1 MYC TP53
5 11.92 ATM MYC TP53
6 11.84 ATM MLH1 TP53
7 11.81 ATM MYC MYCN TP53
8 11.77 CD38 CD5 NOTCH1
9 11.75 MYC NOTCH1 TP53
10 11.73 ATM MYC TP53
11 11.71 IRF4 MYC TP53
12 11.47 ATM MLH1 TP53
13 11.24 ATM MYC TP53
14 11.16 ATM MYC TP53
15 11.15 MYC TP53
16 11.12 ATM TP53
17 11.12 ATM MLH1
18 11.07 ATM TP53
19 11.06 MYC TP53
20 11.04 MYC TP53
21 10.98 ATM MYC TP53
22 10.91 MYC TP53
23 10.84 ATM MYC MYCN NOTCH1 TP53
24 10.83 ATM TP53
25
Show member pathways
10.47 ATM MYC TP53
26 10.1 MYC MYCN

GO Terms for Richter's Syndrome

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

(show all 17)
id Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription from RNA polymerase II promoter GO:0045944 9.85 IRF4 MYC MYCN NOTCH1 TP53
2 cellular response to DNA damage stimulus GO:0006974 9.73 ATM MLH1 MYC TP53
3 positive regulation of gene expression GO:0010628 9.62 CRP MYC MYCN TP53
4 cell cycle arrest GO:0007050 9.58 ATM MYC TP53
5 response to gamma radiation GO:0010332 9.57 MYC TP53
6 negative regulation of fibroblast proliferation GO:0048147 9.56 MYC TP53
7 branching morphogenesis of an epithelial tube GO:0048754 9.55 MYCN NOTCH1
8 reciprocal meiotic recombination GO:0007131 9.54 ATM MLH1
9 positive regulation of DNA binding GO:0043388 9.52 IRF4 MYC
10 positive regulation of mesenchymal cell proliferation GO:0002053 9.51 MYC MYCN
11 cellular response to gamma radiation GO:0071480 9.49 ATM TP53
12 male meiosis GO:0007140 9.48 ATM MLH1
13 determination of adult lifespan GO:0008340 9.37 ATM TP53
14 replicative senescence GO:0090399 9.26 ATM TP53
15 meiotic telomere clustering GO:0045141 9.16 ATM MLH1
16 positive regulation of transcription, DNA-templated GO:0045893 9.1 CD38 IRF4 MYC MYCN NOTCH1 TP53
17 regulation of auditory receptor cell differentiation GO:0045607 8.96 MYCN NOTCH1

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

id Name GO ID Score Top Affiliating Genes
1 protein dimerization activity GO:0046983 9.13 ATM MYC MYCN
2 transcription factor activity, sequence-specific DNA binding GO:0003700 9.02 IRF4 MYC MYCN NOTCH1 TP53

Sources for Richter's Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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