MCID: RCH001
MIFTS: 45

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 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 penile disease. An important gene associated with Richter's Syndrome is CD5 (CD5 Molecule), and among its related pathways/superpathways are Apoptosis Modulation and Signaling and Pathways in cancer. The drugs Cyclophosphamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include b cells, lymph node and liver, and related phenotypes are Synthetic lethal with MLN4924 (a NAE inhibitor) and cellular

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 66)
id Related Disease Score Top Affiliating Genes
1 kotzot-richter syndrome 11.8
2 penile disease 10.6 CD5 IRF4
3 childhood spinal cord tumor 10.6 CD5 TP53
4 congenital sucrase-isomaltase deficiency without starch intolerance 10.6 NOTCH1 TP53
5 metabolic acidosis 10.6 CD38 CD5
6 thai symphalangism syndrome 10.5 NOTCH1 TP53
7 arteriosclerosis 10.5 CD38 CD5
8 rete testis adenoma 10.5 NOTCH1 TP53
9 uterine ligament endometrioid adenocarcinoma 10.5 CD5 MYC
10 phalanx chondroma 10.5 NOTCH1 TP53
11 plummer vinson syndrome 10.5 CD38 NOTCH1
12 chest wall lymphoma 10.4 ATM TP53
13 plasmodium ovale malaria 10.4 CD5 TP53
14 brenner tumor of the vagina 10.4 ATM TP53
15 otitis externa 10.4 MYC TP53
16 primary malignant melanoma of the conjunctiva 10.4 IRF4 MYC
17 brenner tumor of ovary 10.4 ATM TP53
18 villous adenocarcinoma 10.4 MYC TP53
19 rectum squamous cell carcinoma 10.4 MLH1 TP53
20 prostate adenoid cystic carcinoma 10.3 NOTCH1 TP53
21 estrogen-receptor positive breast cancer 10.3 CD5 MYC
22 biliary dyskinesia 10.3 ATM TP53
23 chronic lymphocytic leukemia 10.3
24 leukemia 10.3
25 malignant melanocytic peripheral nerve sheath tumor of mediastinum 10.2 CD38 MYC TP53
26 acral lentiginous melanoma 10.2 CD38 MYC TP53
27 angiomatous meningioma 10.2 CD38 MYC
28 nuchal bleb, familial 10.1 BCR CD5
29 lymphoma, small cleaved-cell, diffuse 10.1 BCR CD5
30 lymphoma 10.1
31 familial episodic pain syndrome 10.1 MLH1 TP53
32 vulvar keratoacanthoma-like carcinoma 10.1 MYC TP53
33 cd3delta deficiency 10.1 CD5 NOTCH1
34 b-cell lymphomas 10.0
35 trachea squamous cell carcinoma 10.0 MLH1 MYC TP53
36 bardet-biedl syndrome 10.0 MLH1 MYC TP53
37 mild hyperphenylalaninemia 10.0 ATM MYC
38 gallbladder cancer 10.0 MLH1 MYC TP53
39 nail disorder, nonsyndromic congenital, 1 10.0 MLH1 MYC TP53
40 arrhythmogenic right ventricular cardiomyopathy 10.0 MLH1 MYC TP53
41 gastric cancer, somatic 10.0 MLH1 MYC TP53
42 diffuse large b-cell lymphoma 10.0
43 drug-induced hepatitis 10.0 CD5 IRF4 MYC TP53
44 bipolar disorder 10.0 CD5 IRF4 MYC TP53
45 bagatelle cassidy syndrome 10.0 CD38 CD5 MYC TP53
46 nasopharyngeal carcinoma 10.0 ATM MLH1 TP53
47 cpt deficiency, hepatic, type ia 9.9 CD38 IRF4 MYC TP53
48 hepatic osteogenic sarcoma 9.9 ATM CD38 CD5 TP53
49 immunodeficiency 11a 9.9 BCR CD38 CD5
50 pneumonic plague 9.8 ATM CD38 CD5 MYC

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 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-1 9.32 MLH1 TP53 ATM ESPL1
2 Synthetic lethal with MLN4924 (a NAE inhibitor) GR00250-A-2 9.32 MYC TP53 ATM CD38 ESPL1 MLH1

MGI Mouse Phenotypes related to Richter's Syndrome:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.03 ESPL1 MLH1 MYC NOTCH1 TP53 ATM
2 hematopoietic system MP:0005397 9.97 ATM BCR CD38 CD5 ESPL1 IRF4
3 endocrine/exocrine gland MP:0005379 9.91 ATM CD38 ESPL1 MLH1 MYC NOTCH1
4 immune system MP:0005387 9.91 MYC NOTCH1 TP53 ATM BCR CD38
5 embryo MP:0005380 9.8 ATM ESPL1 MYC NOTCH1 TP53
6 integument MP:0010771 9.63 ATM CD5 MLH1 MYC NOTCH1 TP53
7 neoplasm MP:0002006 9.5 ATM ESPL1 IRF4 MLH1 MYC NOTCH1
8 normal MP:0002873 9.02 BCR CD5 MYC NOTCH1 TP53

Drugs & Therapeutics for Richter's Syndrome

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

(show top 50) (show all 65)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
2
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
3
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
4
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
5
Cytarabine Approved, Investigational Phase 1, Phase 2 147-94-4 6253
6
Fludarabine Approved Phase 1, Phase 2 21679-14-1, 75607-67-9 30751
7
Oxaliplatin Approved, Investigational Phase 1, Phase 2 61825-94-3 5310940 9887054 43805 6857599
8
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
9
Vidarabine Approved Phase 1, Phase 2 24356-66-9 32326 21704
10
Lenalidomide Approved Phase 2,Phase 1 191732-72-6 216326
11
Etoposide Approved Phase 2 33419-42-0 36462
12
Obinutuzumab Approved Phase 2,Phase 1 949142-50-1
13
Pembrolizumab Approved Phase 2,Phase 1 1374853-91-4
14
Blinatumomab Approved Phase 2 853426-35-4
15
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
16
Everolimus Approved Phase 1, Phase 2 159351-69-6 6442177
17
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 5284616 6436030 46835353
18
nivolumab Approved Phase 1, Phase 2 946414-94-4
19
Idelalisib Approved Phase 2 870281-82-6
20
Asparaginase Approved Phase 2 9015-68-3
21
Azacitidine Approved, Investigational Phase 2 320-67-2 9444
22
Doxil Approved June 1999 Phase 2 31703
23 Antibodies Phase 2,Phase 1
24 Antibodies, Monoclonal Phase 2,Phase 1
25 Immunoglobulins Phase 2,Phase 1
26 Anti-Infective Agents Phase 1, Phase 2
27 Antimetabolites Phase 1, Phase 2
28 Antimetabolites, Antineoplastic Phase 1, Phase 2
29 Antirheumatic Agents Phase 1, Phase 2
30 Antiviral Agents Phase 1, Phase 2
31 Immunosuppressive Agents Phase 1, Phase 2
32 Alkylating Agents Phase 2
33 Anti-Bacterial Agents Phase 2,Phase 1
34 Antibiotics, Antitubercular Phase 2
35 Anti-Inflammatory Agents Phase 2,Phase 1
36 Antimitotic Agents Phase 2
37 Antineoplastic Agents, Hormonal Phase 2,Phase 1
38 Antineoplastic Agents, Phytogenic Phase 2
39 glucocorticoids Phase 2,Phase 1
40 Hormone Antagonists Phase 2,Phase 1
41 Hormones Phase 2,Phase 1
42 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2,Phase 1
43 Topoisomerase Inhibitors Phase 2
44 Angiogenesis Inhibitors Phase 2,Phase 1
45 Angiogenesis Modulating Agents Phase 2,Phase 1
46 Etoposide phosphate Phase 2
47 Antibodies, Bispecific Phase 2
48 Antiemetics Phase 2,Phase 1
49 Autonomic Agents Phase 2,Phase 1
50 BB 1101 Phase 2

Interventional clinical trials:

(show all 35)

id Name Status NCT ID Phase Drugs
1 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 ACP-196;ibrutinib
2 Study of Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone (CHOP) With Ofatumumab in Patients With Richter's Syndrome Unknown status NCT01171378 Phase 2 Ofatumumab
3 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
4 Oxaliplatin, Fludarabine, Cytarabine, and Rituximab in Patients With Richter's Transformation and Leukemias Completed NCT00472849 Phase 1, Phase 2 Oxaliplatin;Fludarabine;Cytarabine;Rituximab;Pegfilgrastim
5 Oxaliplatin, Fludarabine, Cytarabine and Rituximab in Richter's Syndrome, Refractory CLL and PLL Completed NCT00452374 Phase 1, Phase 2 Cytarabine;Fludarabine;Oxaliplatin;Rituximab
6 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
7 Efficacy and Safety Study of PCI-32765 Combine With Ofatumumab in CLL Completed NCT01217749 Phase 1, Phase 2 PCI-32765;ofatumumab
8 A Phase II Study of Venetoclax in Combination With Dose-adjusted EPOCH-R for Patients With Richter's Syndrome Recruiting NCT03054896 Phase 2 Venetoclax
9 Study of Ibrutinib & Obinutuzumab With/Without CHOP for Richter's Transformation or Richter's Syndrome Patients Recruiting NCT03145480 Phase 2 Obinutuzumab;Ibrutinib
10 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
11 Study of Blinatumomab in Richter Transformation Recruiting NCT03121534 Phase 2 Blinatumomab;Dexamethasone
12 Obinutuzumab Containing Conditioning Regimen for Patients With Poor Risk CLL or Richter`s Transformation Requiring Allogeneic Stem Cell Transplantation Recruiting NCT03153514 Phase 2
13 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
14 A Study of Acalabrutinib and Vistusertib in Subjects With Relapsed/Refractory B-cell Malignancies Recruiting NCT03205046 Phase 1, Phase 2 acalabrutinib;vistusertib
15 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 lenalidomide
16 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 Ibrutinib;Nivolumab
17 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
18 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
19 Trial of Ibrutinib Plus Venetoclax Plus Obinutuzumab in Patients With CLL Recruiting NCT02758665 Phase 2 ibrutinib;obinutuzumab;venetoclax
20 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
21 ACP-196 (Acalabrutinib) in Combination With Pembrolizumab, for Treatment of Hematologic Malignancies Active, not recruiting NCT02362035 Phase 1, Phase 2 Acalabrutinib;Pembrolizumab
22 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 Fludarabine;Cytarabine;Erwinase
23 Selinexor in Initial or Relapsed/Refractory Richter's Transformation Terminated NCT02138786 Phase 2 selinexor
24 PNT2258 for Treatment of Patients With Richter's Transformation (Brighton) Terminated NCT02378038 Phase 2 PNT2258
25 5-Azacytidine (Azacytidine; Vidaza) in Chronic Lymphocytic Leukemia Terminated NCT00413478 Phase 2 5-Azacytidine
26 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
27 Lenalidomide After Donor Bone Marrow Transplant in Treating Patients With High-Risk Hematologic Cancers Completed NCT01254578 Phase 1 Lenalidomide
28 Obinutuzumab, High Dose Methylprednisolone (HDMP), and Lenalidomide for the Treatment of Patients With Richter's Syndrome Recruiting NCT03113695 Phase 1 Obinutuzumab;lenalidomide;HDMP
29 Study of AZD5991 in Relapsed or Refractory Haematologic Malignancies. Recruiting NCT03218683 Phase 1 AZD5991;AZD5991;AZD5991;AZD5991;AZD5991
30 Study to Evaluate Safety and Tolerability of XmAb13676 in Patients With CD20-expressing Hematologic Malignancies Recruiting NCT02924402 Phase 1
31 Study to Assess Safety, Tolerability, Pharmacokinetics and Antitumor Activity of AZD4573 in Relapsed/Refractory Haematological Malignancies Not yet recruiting NCT03263637 Phase 1 AZD4573
32 Phase 1 Study Evaluating ZEN003365 in Relapsed/Refractory Lymphoproliferative Malignancies or Relapsed/Refractory AML Withdrawn NCT02238522 Phase 1 ZEN003365
33 Registry of the German CLL Study Group Recruiting NCT02863692
34 Identification of Biomarkers That Are Predictive of Early Ibrutinib Treatment Failure in High Risk TP53 Mutated Chronic Lymphocytic Leukemia Recruiting NCT02827617 Ibrutinib
35 Clinical-biological Characteristics and Outcome of Chronic Lymphocytic Leukemia Under Ibrutinib-named Patient Program Recruiting NCT02582320 Ibrutinib

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
B Cells, Lymph Node, Liver, Spleen, Bone, Bone Marrow, Brain

Publications for Richter's Syndrome

Articles related to Richter's Syndrome:

(show top 50) (show all 138)
id Title Authors Year
1
Richter's Syndrome with Hypercalcemia Induced by Tumor-Associated Production of Parathyroid Hormone-Related Peptide. ( 28203174 )
2017
2
Intravascular large B-cell lymphoma presenting as Richter's syndrome with cerebral involvement in a patient with chronic lymphocytic leukemia. ( 28878900 )
2017
3
Ibrutinib-induced rapid response in chemotherapy-refractory Richter's syndrome. ( 28681549 )
2017
4
Richter's syndrome: Novel and promising therapeutic alternatives. ( 27742070 )
2016
5
EBV-positive Richter's syndrome with laboratory features of Burkitt's lymphoma, in Ibrutinib-treated chronic lymphocytic leukemia. ( 27852142 )
2016
6
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
7
An 18F-FDG-PET maximum standardized uptake value >10 represents a novel valid marker for discerning Richter's Syndrome. ( 26402256 )
2015
8
Chronic lymphocytic leukemia transformation into high-grade lymphoma: a description of Richter's syndrome in eight dogs. ( 26463596 )
2015
9
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
10
Ibrutinib has some activity in Richter's syndrome. ( 25635530 )
2015
11
Cutaneous Richter's syndrome: A better place to transform? ( 24935194 )
2014
12
Hemophagocytic Lymphohistiocytosis With Cerebral Involvement in Richter's Syndrome. ( 25534389 )
2014
13
Testicular swelling: a rare manifestation of chronic lymphocytic leukemia presenting with Richter's syndrome. ( 24739853 )
2014
14
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
15
Richter's syndrome: Utility of A^a8,F-FDG PET/CT. ( 23044071 )
2013
16
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
17
Richter's syndrome: CLL taking a turn for the worse. ( 23413593 )
2012
18
Richter's syndrome with aberrant CD2 and CD4 expression. ( 22649134 )
2012
19
Richter's syndrome in colon: case report. ( 21057893 )
2012
20
MDR-1 gene polymorphisms G2677T and C3435T in a case of Hodgkin's variant of Richter's syndrome. ( 22866091 )
2011
21
Richter's syndrome of the brain and the spinal cord. ( 20587487 )
2011
22
Haemophagocytic syndrome and Hodgkin's disease variant of Richter's syndrome after fludarabine for CLL. ( 20331737 )
2010
23
Hodgkin's lymphoma variant of Richter's syndrome. ( 20437646 )
2010
24
Genomic profiling of Richter's syndrome: recurrent lesions and differences with de novo diffuse large B-cell lymphomas. ( 20014148 )
2010
25
Cutaneous Richter's syndrome, prognosis, and clinical, histological and immunohistological patterns: report of four cases and review of the literature. ( 20145381 )
2010
26
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
27
Telomere length is an independent predictor of survival, treatment requirement and Richter's syndrome transformation in chronic lymphocytic leukemia. ( 19340005 )
2009
28
Richter's syndrome developing in a patient with adult onset Still's disease. ( 18607590 )
2009
29
Richter's syndrome: a case report. ( 19204436 )
2009
30
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
31
[Clonality analysis and mutation status of IgVH genes in classic Richter's syndrome]. ( 19031714 )
2008
32
Recent advances in the diagnosis and therapy of Richter's syndrome. ( 17673808 )
2007
33
Primary effusion lymphoma presenting as Richter's syndrome. ( 17573910 )
2007
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
Clinical outcomes and prognostic factors in patients with Richter's syndrome treated with chemotherapy or chemoimmunotherapy with or without stem-cell transplantation. ( 16710033 )
2006
36
Chronic lymphocytic leukemia with osteolytic Richter's syndrome mimicking myeloma bone disease shows no over-expression of DKK1. ( 17065022 )
2006
37
EBV negative Richter's syndrome from a coexistent clone after salvage treatment with alemtuzumab in a CLL patient. ( 16838338 )
2006
38
[Isolated Richter's syndrome in central nervous system: case report]. ( 16059612 )
2005
39
Hodgkin's disease variant of Richter's syndrome in chronic lymphocytic leukaemia patients previously treated with fludarabine. ( 15813847 )
2005
40
Cat scratch disease mimicking Richter's Syndrome in a patient with chronic lymphocytic leukemia. ( 15621836 )
2005
41
Richter's syndrome: biology and therapy. ( 16053658 )
2005
42
Richter's syndrome--the downside of fludarabine? ( 16111529 )
2005
43
Unusual sites of malignancies: CASE 2. Central neurogenic hyperventilation as a complication of Richter's syndrome. ( 15774798 )
2005
44
Second malignancies and Richter's syndrome in patients with chronic lymphocytic leukemia. ( 15763979 )
2004
45
Second malignancies and Richter's syndrome in patients with chronic lymphocytic leukaemia treated with cladribine. ( 14746857 )
2004
46
Relationship between the mutational status of VH genes and pathogenesis of diffuse large B-cell lymphoma in Richter's syndrome. ( 14671632 )
2004
47
Richter's syndrome in the brain first manifested as an ischaemic stroke. ( 15360010 )
2004
48
Richter's syndrome presenting as a nasal lymphoma. ( 15223655 )
2004
49
Distinct clonal origin in two cases of Hodgkin's lymphoma variant of Richter's syndrome associated With EBV infection. ( 15105659 )
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

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 28)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.6 ATM IRF4 MYC TP53
2 12.42 BCR MLH1 MYC TP53
3
Show member pathways
12.3 ATM MLH1 MYC NOTCH1 TP53
4 12.21 ATM MYC NOTCH1 TP53
5 12.11 CD38 MYC TP53
6 12.04 ATM MYC TP53
7 12.03 ATM ESPL1 MYC TP53
8 11.98 MLH1 MYC TP53
9 11.93 ATM MYC TP53
10 11.84 ATM MLH1 TP53
11 11.75 CD38 CD5 NOTCH1
12 11.74 MYC NOTCH1 TP53
13 11.71 ATM MYC TP53
14 11.69 IRF4 MYC TP53
15 11.44 ATM MLH1 TP53
16 11.2 ATM MYC TP53
17 11.16 MYC TP53
18 11.14 ATM TP53
19 11.13 ATM MLH1
20 11.1 ATM MYC TP53
21 11.08 ATM TP53
22 11.05 MYC TP53
23 11.01 CPT2 MYC
24 10.98 ATM MYC TP53
25 10.92 MYC TP53
26 10.84 ATM TP53
27 10.74 ATM MYC NOTCH1 TP53
28
Show member pathways
10.47 ATM MYC TP53

GO Terms for Richter's Syndrome

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

(show all 13)
id Name GO ID Score Top Affiliating Genes
1 cellular response to DNA damage stimulus GO:0006974 9.73 ATM MLH1 MYC TP53
2 cell cycle arrest GO:0007050 9.58 ATM MYC TP53
3 response to gamma radiation GO:0010332 9.52 MYC TP53
4 negative regulation of fibroblast proliferation GO:0048147 9.51 MYC TP53
5 reciprocal meiotic recombination GO:0007131 9.49 ATM MLH1
6 male meiosis GO:0007140 9.48 ATM MLH1
7 cellular response to gamma radiation GO:0071480 9.46 ATM TP53
8 determination of adult lifespan GO:0008340 9.37 ATM TP53
9 replicative senescence GO:0090399 9.32 ATM TP53
10 meiotic telomere clustering GO:0045141 9.16 ATM MLH1
11 positive regulation of transcription, DNA-templated GO:0045893 9.02 CD38 IRF4 MYC NOTCH1 TP53
12 positive regulation of response to DNA damage stimulus GO:2001022 8.96 MYC
13 homologous chromosome segregation GO:0045143 8.96 ESPL1 MLH1

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 HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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