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MCID: RCH001
MIFTS: 45

Richter's Syndrome

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases
Genes Variations Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Richter's Syndrome

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MalaCards integrated aliases for Richter's Syndrome:

Name: Richter's Syndrome 12 15 71
Richter Syndrome 12 74 52
Richter Transformation 52

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Immune diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 32


External Ids:

Disease Ontology 12 DOID:1703
NCIt 49 C35424
SNOMED-CT 67 277550009
ICD10 32 C91.1
UMLS 71 C0349631
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Summaries for Richter's Syndrome

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NIH Rare Diseases : 52 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 bone lymphoma and plasmablastic lymphoma. An important gene associated with Richter's Syndrome is CD5 (CD5 Molecule), and among its related pathways/superpathways are Endometrial cancer and Pathways in cancer. The drugs Arsenic trioxide and ofatumumab have been mentioned in the context of this disorder. Affiliated tissues include b cells, myeloid and lymph node, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

Wikipedia : 74 Richter's syndrome (RS), also known as Richter's transformation, is a transformation of B cell chronic... more...

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Related Diseases for Richter's Syndrome

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

(show top 50) (show all 250)
# Related Disease Score Top Affiliating Genes
1 bone lymphoma 30.4 SPN IRF4 CD5
2 plasmablastic lymphoma 30.4 NOTCH1 MYC CD38
3 adult t-cell leukemia 30.2 TP53 MYC IRF4
4 peripheral t-cell lymphoma 30.2 TP53 NOTCH1 CD5 CD38
5 diffuse large b-cell lymphoma 30.0 TP53 SPN MYC MIR17 IRF4 CD5
6 leukemia 30.0 TP53 NOTCH1 MYC CSF2 BCR ATM
7 lymphoma 30.0 TP53 NOTCH1 MYC IRF4 CSF2 CD5
8 composite lymphoma 29.9 SPN IGHV4-38-2 CD5
9 follicular lymphoma 29.8 MYC MIR17 IRF4 CSF2 CD5
10 lymphoplasmacytic lymphoma 29.7 ZAP70 IRF4 CD5
11 leukemia, chronic lymphocytic 2 29.6 ZAP70 CD5 CD38 ATM
12 burkitt lymphoma 29.5 TP53 MYC IRF4 BCR ATM
13 marginal zone b-cell lymphoma 29.5 SPN IRF4 IGHV4-38-2 CD5 CD38
14 myelodysplastic syndrome 29.4 TP53 NOTCH1 MYC CSF2 CD38 ATM
15 mantle cell lymphoma 29.4 TP53 SPN NOTCH1 MYC MIR17 CD5
16 prolymphocytic leukemia 29.3 ZAP70 TP53 MYC CD5 CD38 ATM
17 anemia, autoimmune hemolytic 29.3 ZAP70 IGHV4-38-2 CD5
18 lung cancer susceptibility 3 29.2 TP53 NOTCH1 MYC MIR17 MIR125A ATM
19 t-cell lymphoblastic leukemia/lymphoma 29.1 ZAP70 NOTCH1 MYC MIR17 IRF4
20 b-cell lymphoma 29.0 ZAP70 TP53 MYC MIR17 IRF4 CD5
21 cll/sll 28.9 ZAP70 TP53 SPN IGHV4-38-2 CD5 CD38
22 cervical cancer 28.2 TP53 NOTCH1 MYC MLH1 MIR181B1 MIR17
23 myeloma, multiple 27.7 TP53 NOTCH1 MYC MIR17 MIR125A IRF4
24 lymphoma, non-hodgkin, familial 27.0 TP53 SPN NOTCH1 MYCN MYC MIR17
25 leukemia, acute myeloid 26.6 TP53 SPN NOTCH1 MYCN MYC MIR17
26 leukemia, chronic lymphocytic 26.6 ZAP70 TP53 SPN NOTCH1 MYC MIR181B1
27 leukemia, acute lymphoblastic 25.7 ZAP70 TP53 NOTCH1 MYCN MYC MIR17
28 kotzot-richter syndrome 12.3
29 splenic diffuse red pulp small b-cell lymphoma 10.5 TP53 NOTCH1
30 primary cutaneous diffuse large b-cell lymphoma, leg type 10.5 MYC IRF4
31 bladder lymphoma 10.5 SPN CD5
32 endocrine exophthalmos 10.4 CD5 ATM
33 anal carcinoma in situ 10.4 TP53 MYC
34 lung lymphoma 10.4 SPN CD5
35 lymphoma, hodgkin, classic 10.4
36 appendix lymphoma 10.4 MYC CD5
37 t-cell adult acute lymphocytic leukemia 10.4 TP53 IRF4 CD5
38 adult lymphoma 10.4 MYC IRF4 CD5
39 breast lymphoma 10.4 SPN IRF4 CD5
40 maxillary sinus adenocarcinoma 10.4 TP53 ATM
41 cerebral primitive neuroectodermal tumor 10.4 MYCN MYC
42 suppressor of tumorigenicity 3 10.3 TP53 MYC
43 lung adenoma 10.3 TP53 NOTCH1 MYC
44 gastrointestinal lymphoma 10.3 SPN CD5
45 cryoglobulinemia, familial mixed 10.3 CD5 BCR
46 actinic keratosis 10.3 TP53 MYC IRF4
47 frontal lobe neoplasm 10.3 TP53 MYCN MYC
48 nodular medulloblastoma 10.3 MYCN MYC
49 myasthenic syndrome, congenital, 17 10.3 TP53 LRP4
50 macroglobulinemia 10.3

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



Diseases related to Richter's Syndrome
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Symptoms & Phenotypes for Richter's Syndrome

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

26 (show all 16)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-104 9.58 MLH1
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-120 9.58 MYCN
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-130 9.58 MLH1
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-138 9.58 CSF2 MLH1 NOTCH1
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-139 9.58 MLH1
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-170 9.58 CSF2
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-190 9.58 CSF2
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-205 9.58 NOTCH1
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-214 9.58 NOTCH1
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-40 9.58 CSF2
11 Decreased shRNA abundance (Z-score < -2) GR00366-A-65 9.58 NOTCH1
12 Decreased shRNA abundance (Z-score < -2) GR00366-A-73 9.58 NOTCH1
13 Decreased shRNA abundance (Z-score < -2) GR00366-A-74 9.58 MLH1
14 Decreased shRNA abundance (Z-score < -2) GR00366-A-75 9.58 NOTCH1
15 Decreased shRNA abundance (Z-score < -2) GR00366-A-88 9.58 MLH1
16 Decreased shRNA abundance (Z-score < -2) GR00366-A-95 9.58 CSF2

MGI Mouse Phenotypes related to Richter's Syndrome:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.27 APOE ATM BCR CD38 CSF2 IRF4
2 hematopoietic system MP:0005397 10.21 APOE ATM BCR CD38 CD5 CSF2
3 immune system MP:0005387 10.13 APOE ATM BCR CD38 CD5 CSF2
4 endocrine/exocrine gland MP:0005379 10.1 APOE ATM CD38 CSF2 LRP4 MLH1
5 digestive/alimentary MP:0005381 9.98 APOE BCR MLH1 MYC MYCN NOTCH1
6 integument MP:0010771 9.96 APOE ATM CD5 CSF2 LRP4 MLH1
7 neoplasm MP:0002006 9.76 ATM CSF2 IRF4 MLH1 MYC NOTCH1
8 renal/urinary system MP:0005367 9.5 APOE BCR CSF2 LRP4 MYCN NOTCH1
9 respiratory system MP:0005388 9.23 APOE CSF2 LRP4 MLH1 MYCN NOTCH1
Sources

Drugs & Therapeutics for Richter's Syndrome

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

(show top 50) (show all 55)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Arsenic trioxide Approved, Investigational Phase 3 1327-53-3 518740
2
ofatumumab Approved Phase 2 679818-59-8 6918251
3
Oxaliplatin Approved, Investigational Phase 1, Phase 2 61825-94-3 43805 6857599 5310940 9887054
4
Cytarabine Approved, Experimental, Investigational Phase 1, Phase 2 147-94-4, 65-46-3 6253
5
Atezolizumab Approved, Investigational Phase 2 1380723-44-3
6
Zanubrutinib Approved, Investigational Phase 2 1691249-45-2
7
Fludarabine Approved Phase 2 21679-14-1, 75607-67-9 30751
8
Blinatumomab Approved, Investigational Phase 2 853426-35-4
9
Venetoclax Approved, Investigational Phase 2 1257044-40-8 49846579
10
nivolumab Approved Phase 2 946414-94-4
11
Pembrolizumab Approved Phase 2 1374853-91-4
12
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
13
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
14
rituximab Approved Phase 2 174722-31-7 10201696
15
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
16
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
17
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
18
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
19 Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
20
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
21
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
22
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
23
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
24
Obinutuzumab Approved, Investigational Phase 2 949142-50-1
25
Tyrosine Approved, Investigational, Nutraceutical Phase 1, Phase 2 60-18-4 6057
26
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
27 Anti-Infective Agents Phase 1, Phase 2
28 Antiviral Agents Phase 1, Phase 2
29 Antimetabolites Phase 1, Phase 2
30 Immunoglobulins Phase 2
31 Antibodies Phase 2
32 Antibodies, Monoclonal Phase 2
33 Protein Kinase Inhibitors Phase 2
34 Antiemetics Phase 2
35 Gastrointestinal Agents Phase 2
36 BB 1101 Phase 2
37 AZD5991 Phase 1, Phase 2
38 Immunologic Factors Phase 2
39 Hormones Phase 2
40 Antineoplastic Agents, Hormonal Phase 2
41 Hormone Antagonists Phase 2
42 glucocorticoids Phase 2
43 Methylprednisolone Acetate Phase 2
44 Immunosuppressive Agents Phase 2
45 Tubulin Modulators Phase 2
46 Alkylating Agents Phase 2
47 Anti-Bacterial Agents Phase 2
48 Antibiotics, Antitubercular Phase 2
49 Antirheumatic Agents Phase 2
50 Antimitotic Agents Phase 2

Interventional clinical trials:

(show all 29)
# Name Status NCT ID Phase Drugs
1 Combination of Ibrutinib and As2O3 in the Treatment of CLL Unknown status NCT02757040 Phase 3 Ibrutinib combined with As2O3;ibrutinib
2 A Phase I/II Study of Cloretazine in Patients With Refractory/Relapsed Chronic Lymphocytic Leukemia or Richter's Syndrome Completed NCT00304005 Phase 1, Phase 2 laromustine
3 CHOP Plus Rituximab (CHOP-R) in Fludarabine Refractory Chronic Lymphocytic Leukemia (CLL) or CLL With Autoimmune Haemolytic Anemia (AIHA) or Richter's Transformation (RT) Completed NCT00309881 Phase 2 cyclophosphamide;doxorubicin hydrochloride;prednisone;vincristine sulfate
4 Single Arm NCRI Feasibility Study of CHOP in Combination With Ofatumumab in Induction and Maintenance for Patients With Newly Diagnosed Richter's Syndrome Completed NCT01171378 Phase 2 Ofatumumab
5 A Phase I-II Study of Oxaliplatin, Fludarabine, Cytarabine, and Rituximab in Patients With Richter's Transformation, Prolymphocytic Leukemia, Aggressive, Relapsed or Refractory B-Cell Chronic Lymphocytic Leukemia Completed NCT00472849 Phase 1, Phase 2 Oxaliplatin;Fludarabine;Cytarabine;Rituximab;Pegfilgrastim
6 BLINAtumomab After R-CHOP Debulking Therapy for Patients With Richter Transformation Recruiting NCT03931642 Phase 2 RCHOP;Blinatumomab
7 Atezolizumab (PD-L1 mAb) in Combination With Obinutuzumab and Venetoclax for Patients With Chronic Lymphocytic Leukemia and Richter Transformation Recruiting NCT02846623 Phase 2 Atezolizumab;Venetoclax
8 A Prospective, Open-label, Multicenter Phase-II Trial to Evaluate the Efficacy and Safety of Zanubrutinib (BGB-3111), a BTK Inhibitor, Plus Tislelizumab (BGB-A317), a PD1 Inhibitor, for Treatment of Patients With Richter Transformation Recruiting NCT04271956 Phase 2 Zanubrutinib
9 A Multi-Center, Open Label, Uncontrolled, Phase II Clinical Trial Evaluating the Safety and Efficacy of Venetoclax in Combination With Atezolizumab and Obinutuzumab in Richter Transformation of CLL Recruiting NCT04082897 Phase 2 Obinutuzumab 25 MG/ML [Gazyva];Atezolizumab 60 MG/ML [Tecentriq];Venetoclax Oral Tablet
10 A Phase I/II Study of Duvelisib and Venetoclax in Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia or Small Lymphocytic Lymphoma or Patients With Richter's Syndrome Recruiting NCT03534323 Phase 1, Phase 2 Duvelisib;Venetoclax
11 Duvelisib for Ibrutinib-Resistant Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) Recruiting NCT04209621 Phase 2 Duvelisib;Ibrutinib
12 Evaluation of Risk-Adapted and MRD-Driven Strategy for Untreated Fit Patients With Intermediate Risk Chronic Lymphocytic Leukemia: a Randomized Phase II Trial Comparing FCR and a Chemo-free Combination of Venetoclax and Ibrutinib Recruiting NCT04010968 Phase 2 venetoclax and ibrutinib (I+VEN);FCR
13 Venetoclax Plus Dose-adjusted EPOCH-R for Richter's Syndrome Active, not recruiting NCT03054896 Phase 2 Venetoclax
14 A Phase 1/2, Multicenter, Open-label, and Dose-escalation Study of ACP-196 in Subjects With Chronic Lymphocytic Leukemia, Richter's Syndrome or Prolymphocytic Leukemia Active, not recruiting NCT02029443 Phase 1, Phase 2 acalabrutinib
15 Nivolumab Combined With Ibrutinib for Relapsed, Refractory or High-Risk Untreated Patients With Chronic Lymphocytic Leukemia (CLL) Active, not recruiting NCT02420912 Phase 2 Ibrutinib
16 A Phase II Study of Pembrolizumab (MK-3475) in Subjects With Relapsed or Refractory Primary Mediastinal Large B-cell Lymphoma (rrPMBCL) or Relapsed or Refractory Richter Syndrome (rrRS) Active, not recruiting NCT02576990 Phase 2
17 A Phase II Study of Blinatumomab in Richter Transformation Active, not recruiting NCT03121534 Phase 2 Blinatumomab;Dexamethasone
18 A Phase 1/2a Study to Evaluate the Safety, Pharmacokinetics, Pharmacodynamics, and Preliminary Efficacy of the Combination of Ibrutinib With Nivolumab in Subjects With Hematologic Malignancies Active, not recruiting NCT02329847 Phase 1, Phase 2 Ibrutinib;Nivolumab
19 A Phase 1/1b/2a, 3-Part, Open-Label, Multicentre Study to Assess the Safety, Tolerability, Pharmacokinetics and Preliminary Antitumor Activity of Ascending Doses of AZD5991 in Subjects With Relapsed or Refractory Haematologic Malignancies Active, not recruiting NCT03218683 Phase 1, Phase 2 AZD5991;AZD5991 + Venetoclax
20 A Phase II, Randomised Study of CHOP-R in Combination With Acalabrutinib Compared to CHOP-R in Patients With Newly Diagnosed Richter's Syndrome and a Platform for Initial Investigations Into Activity of Novel Treatments in Relapsed/Refractory and Newly Diagnosed Richter's Syndrome. Not yet recruiting NCT03899337 Phase 2 Acalabrutinib;Cyclophosphamide;Doxorubicin;Vincristine;Prednisolone;Rituximab
21 Two-arm Phase II Trial Exploring the Use of the Targeted Agents Ibrutinib and Obinutuzumab for the Treatment of Patients With a Diagnosis of Richter's Transformation (RT) or Richter's Syndrome (RS) Terminated NCT03145480 Phase 2 Obinutuzumab;Ibrutinib
22 A Phase 2 Study of the Safety and Anti-tumor Activity of the Oral Selective Inhibitor of Nuclear Export (SINE) Selinexor (KPT-330) in Patients With Initial or Refractory and/or Relapsed Richter's Transformation (RT) Terminated NCT02138786 Phase 2 selinexor
23 A Phase I Study of Duvelisib in Combination With Nivolumab for Patients With Richter's Syndrome and Transformed Follicular Lymphoma Recruiting NCT03892044 Phase 1 Duvelisib
24 Obinutuzumab, High Dose Methylprednisolone (HDMP), and Lenalidomide for the Treatment of Patients With Richter's Syndrome Recruiting NCT03113695 Phase 1 Obinutuzumab;lenalidomide;HDMP
25 A Phase 1 Dose-Escalation and Cohort-Expansion Study of VLS -101 in Subjects With Hematological Malignancies Recruiting NCT03833180 Phase 1 VLS-101 Schedule 1;VLS-101 Schedule 2;VLS-101 Schedule 3
26 A Phase 1, Open-Label, Multicentre, Non-Randomized Study to Assess the Safety, Tolerability, Pharmacokinetics and Preliminary Antitumor Activity of AZD4573, a Potent and Selective CDK9 Inhibitor, in Subjects With Relapsed or Refractory Haematological Malignancies Active, not recruiting NCT03263637 Phase 1 AZD4573
27 Phase 1 Open-label Dose Escalation and Expansion Study of ZEN003365 in Subjects With Relapsed or Refractory Lymphoproliferative Malignancies or Acute Myeloid Leukemia Withdrawn NCT02238522 Phase 1 ZEN003365
28 Genomic and Proteomic Study of Richter Syndrome Recruiting NCT03619512
29 A Retrospective Study to Evaluate the Clinical-Biologic Characteristics and Outcome of Patients Treated in Italy According to the Ibrutinib-Named Patient Program for Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia Recruiting NCT02582320 Ibrutinib

Search NIH Clinical Center for Richter's Syndrome

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Genetic Tests for Richter's Syndrome

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Anatomical Context for Richter's Syndrome

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MalaCards organs/tissues related to Richter's Syndrome:

40
B Cells, Myeloid, Lymph Node, Spleen, Liver, T Cells, Brain
Sources

Publications for Richter's Syndrome

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Articles related to Richter's Syndrome:

(show top 50) (show all 486)
# Title Authors PMID Year
1
Correction: Bidirectional linkage between the B-cell receptor and NOTCH1 in chronic lymphocytic leukemia and in Richter's syndrome: therapeutic implications. 61
31836851 2020
2
Clinical outcome and prognostic factors of patients with Richter syndrome: real-world study of the Spanish Chronic Lymphocytic Leukemia Study Group (GELLC). 61
32519351 2020
3
Pembrolizumab in relapsed or refractory Richter syndrome. 61
32544980 2020
4
Prognostication in Richter syndrome: context is everything. 61
32420622 2020
5
Allogeneic hematopoietic cell transplantation is an effective treatment for patients with Richter syndrome: A systematic review and meta-analysis. 61
32473105 2020
6
Extranodal NK/T cell lymphoma and lymphomatoid granulomatosis in a patient with chronic lymphocytic leukaemia: Case report for a new perspective on Richter syndrome. 61
32384485 2020
7
Multiple B cell malignancies in patients with chronic lymphocytic leukemia: epidemiology, pathology, and clinical implications. 61
31928278 2020
8
Duodenal stricture secondary to Richter's syndrome. 61
31590994 2020
9
Richter's syndrome of T-cell lineage (T rex lymphoma). 61
31910677 2020
10
A case of T-cell chronic lymphocytic leukemia progressing to Richter syndrome with central nervous system involvement in a dog. 61
32215932 2020
11
Rare case of Richter's syndrome localization in liver and thyroid of a patient with a chronic lymphocytic leukemia (CLL) - Case report and literature. 61
32208597 2020
12
Prognostic Value of Baseline Total Metabolic Tumor Volume Measured on FDG PET in Patients With Richter Syndrome. 61
31876819 2020
13
Bidirectional linkage between the B-cell receptor and NOTCH1 in chronic lymphocytic leukemia and in Richter's syndrome: therapeutic implications. 61
31467429 2020
14
[Sequential development of mantle cell lymphoma following chronic lymphocytic leukemia]. 61
32224586 2020
15
Computed tomography textural analysis for the differentiation of chronic lymphocytic leukemia and diffuse large B cell lymphoma of Richter syndrome. 61
31236702 2019
16
High-grade B-Cell lymphoma with MYC and BCL6 rearrangements associated with Richter transformation of chronic lymphocytic leukemia. 61
31440479 2019
17
Richter syndrome epidemiology in a large population based chronic lymphocytic leukemia cohort from Norway. 61
30986631 2019
18
The clinical study on treatment of CD19-directed chimeric antigen receptor-modified T cells in a case of refractory Richter syndrome. 61
31050207 2019
19
Durable Response to Venetoclax Monotherapy in Richter's Syndrome: A Case Report and Review of Literature. 61
32300445 2019
20
The STELLAR trial protocol: a prospective multicentre trial for Richter's syndrome consisting of a randomised trial investigation CHOP-R with or without acalabrutinib for newly diagnosed RS and a single-arm platform study for evaluation of novel agents in relapsed disease. 61
31109313 2019
21
The involvement of microRNA in the pathogenesis of Richter syndrome. 61
30409799 2019
22
Use of PD-1 (PDCD1) inhibitors for the treatment of Richter syndrome: experience at a single academic centre. 61
30028000 2019
23
BCR kinase inhibitors, idelalisib and ibrutinib, are active and effective in Richter syndrome. 61
29974955 2019
24
Loss of NFAT2 expression results in the acceleration of clonal evolution in chronic lymphocytic leukemia. 61
30556925 2019
25
Richter Syndrome Presenting With Colon Localization. 61
30516680 2019
26
Rapid complete response to blinatumomab as a successful bridge to allogeneic stem cell transplantation in a case of refractory Richter syndrome. 61
29745744 2019
27
Lymphomatoid Granulomatosis in a Patient with Chronic Lymphocytic Leukemia and Rapidly Progressing Peribronchovascular Pulmonary Infiltrates. 61
30805241 2019
28
CD5-negative chronic lymphocytic leukemia/small lymphocytic lymphoma in a patient with gastrointestinal mantle cell lymphoma: an unusual case report. 61
31114236 2019
29
Rapid and complete response to idelalisib in a case of Richter syndrome. 61
30863085 2019
30
Immunochemotherapy for Richter syndrome: current insights. 61
30788335 2019
31
[Richter syndrome successfully treated with ibrutinib monotherapy: two case reports]. 61
31695008 2019
32
Chronic lymphocytic leukemia with proliferation centers in bone marrow is associated with younger age at initial presentation, complex karyotype, and TP53 disruption. 61
30086334 2018
33
Current trends in the management of Richter's syndrome. 61
30651968 2018
34
Richter syndrome: A rare complication of chronic lymphocytic leukemia or small lymphocytic lymphoma. 61
30156387 2018
35
A composite lymphoma combining a Hodgkin lymphoma and a marginal zone lymphoma transformed into a diffuse large B-cell lymphoma. 61
30564326 2018
36
miR-125a and miR-34a expression predicts Richter syndrome in chronic lymphocytic leukemia patients. 61
30242085 2018
37
Exosome-carried microRNA-based signature as a cellular trigger for the evolution of chronic lymphocytic leukemia into Richter syndrome. 61
30238808 2018
38
Extranodal Richter's syndrome of the urinary bladder. 61
30337788 2018
39
Richter's syndrome of the central nervous system diagnosed concurrently with chronic lymphocytic leukaemia: A case report and literature review. 61
30313065 2018
40
Isolated Richter's syndrome of the brain: diagnosis in the eye of the beholder. 61
29627880 2018
41
Novel Richter Syndrome Xenograft Models to Study Genetic Architecture, Biology, and Therapy Responses. 61
29735551 2018
42
A case report of extranodal NK/T-cell lymphoma in patient with chronic lymphocytic leukemia. 61
30045301 2018
43
Biology and treatment of Richter syndrome. 61
29692342 2018
44
Cutaneous Richter Syndrome Mimicking Primary Cutaneous CD4-Positive Small/Medium T-cell Lymphoma: Case Report and Review of the Literature. 61
28937430 2018
45
Treatment of Del17p and/or aberrant TP53 chronic lymphocytic leukemia in the era of novel therapies. 61
28549767 2018
46
Clinical, pathological, and biological characterization of Richter syndrome developing after ibrutinib treatment for relapsed chronic lymphocytic leukemia. 61
29484684 2018
47
Ibrutinib-induced rapid response in chemotherapy-refractory Richter's syndrome. 61
28681549 2018
48
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. 61
29193006 2018
49
Allogeneic Hematopoietic Cell Transplantation for Richter Syndrome: A Single-Center Experience. 61
29126867 2018
50
B-chronic lymphocytic leukemia showed triple transformation, to diffuse large B cell, CD20-negative, and T-cell neoplasm during ofatumumab treatment: a case report. 61
29796007 2018
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Variations for Richter's Syndrome

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Cosmic variations for Richter's Syndrome:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)		 Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM93183630 haematopoietic and lymphoid tissue,lymph node,lymphoid neoplasm,diffuse large B cell lymphoma c.743G>A p.R248Q 17:7674220-7674220 0
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Expression for Richter's Syndrome

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

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

(show all 19)
# Super pathways Score Top Affiliating Genes
1
Endometrial cancer 36
Show member pathways
 12.7 TP53 MYC MLH1 CSF2 BCR
2
Pathways in cancer 36
12.67 TP53 NOTCH1 MYC MLH1 BCR
3
DNA Damage Response 1
Show member pathways
 12.21 TP53 MYC MIR17 ATM
4
Human T-cell leukemia virus 1 infection 36
12.15 TP53 MYC CSF2 ATM
5
Transcriptional misregulation in cancer 36
12 TP53 MYCN MYC CSF2 ATM
6
MicroRNAs in cancer 36
11.99 TP53 NOTCH1 MYC MIR181B1 MIR17 MIR125A
7
Alzheimers Disease 1
11.91 TP53 MIR181B1 MIR125A APOE
8
NF-kB(NFkB) Pathway 66
11.76 ZAP70 BCR ATM
9
Hematopoietic cell lineage 36
11.75 CSF2 CD5 CD38
10
Hematopoietic Stem Cells and Lineage-specific Markers 64
11.62 SPN NOTCH1 CSF2 CD5 CD38
11
Platinum drug resistance 36
11.56 TP53 MLH1 ATM
12
Dendritic Cells Developmental Lineage Pathway 64
11.54 SPN IRF4 CSF2 CD38
13
Integrated Cancer Pathway 1
11.32 TP53 MYC ATM
14
DNA damage_ATM/ATR regulation of G1/S checkpoint 22
11.28 TP53 MYC ATM
15
miRNAs involved in DNA damage response 1
11.26 TP53 MYC MIR181B1 ATM
16
TP53 Network 1
Show member pathways
 10.92 TP53 MYC ATM
17
Pathways Affected in Adenoid Cystic Carcinoma 1
10.84 TP53 NOTCH1 MYCN MYC ATM
18
Development of pulmonary dendritic cells and macrophage subsets 1
10.68 IRF4 CSF2
19
Notch Pathway 70
10.19 MYCN MYC
Sources

GO Terms for Richter's Syndrome

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 cytokine-mediated signaling pathway GO:0019221 9.83 TP53 MYC IRF4 CSF2
2 regulation of cell proliferation GO:0042127 9.71 ZAP70 TP53 NOTCH1 CSF2
3 positive regulation of transcription, DNA-templated GO:0045893 9.7 TP53 NOTCH1 MYCN MYC IRF4 CD38
4 cell cycle arrest GO:0007050 9.56 TP53 NOTCH1 MYC ATM
5 positive regulation of response to DNA damage stimulus GO:2001022 9.51 MYC ATM
6 positive regulation of gene expression GO:0010628 9.5 TP53 NOTCH1 MYCN MYC MIR125A CSF2
7 cardiac septum morphogenesis GO:0060411 9.49 TP53 NOTCH1
8 replicative senescence GO:0090399 9.48 TP53 ATM
9 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.46 TP53 MYCN
10 meiotic telomere clustering GO:0045141 9.43 MLH1 ATM
11 positive regulation of transcription from RNA polymerase II promoter in response to hypoxia GO:0061419 9.37 TP53 NOTCH1
12 lipoprotein catabolic process GO:0042159 9.32 ATM APOE
13 negative regulation of gene expression GO:0010629 9.17 TP53 NOTCH1 MYCN MYC MIR17 MIR125A

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

# Name GO ID Score Top Affiliating Genes
1 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 9.02 TP53 NOTCH1 MYCN MYC IRF4
Sources

Sources for Richter's Syndrome

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
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56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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