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MCID: RCH001
MIFTS: 44

Richter's Syndrome

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases
Genes 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 70
Richter Syndrome 12 73 20
Richter Transformation 20

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 50 C35424
SNOMED-CT 67 277550009
ICD10 32 C91.1
UMLS 70 C0349631
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Summaries for Richter's Syndrome

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GARD : 20 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 merkel cell carcinoma and intravascular large b-cell lymphoma. An important gene associated with Richter's Syndrome is CD5 (CD5 Molecule), and among its related pathways/superpathways are PI3K-Akt signaling pathway and TCR Signaling (Qiagen). The drugs ofatumumab and Pembrolizumab have been mentioned in the context of this disorder. Affiliated tissues include spleen, myeloid and brain, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Wikipedia : 73 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 277)
# Related Disease Score Top Affiliating Genes
1 merkel cell carcinoma 30.5 TP53 NOTCH1 MYC
2 intravascular large b-cell lymphoma 30.3 MYC CD5
3 bone lymphoma 30.2 SPN IRF4 CD5
4 adult t-cell leukemia 30.1 TP53 MYC IRF4
5 plasmablastic lymphoma 30.0 NOTCH1 MYC CD38
6 t-cell lymphoblastic leukemia/lymphoma 30.0 ZAP70 NOTCH1 MYC IRF4
7 composite lymphoma 29.9 IGHV4-38-2 CD5
8 mature t-cell and nk-cell lymphoma 29.7 TP53 MYC MIR17 CD5
9 leukemia, chronic lymphocytic 2 29.6 ZAP70 CD5 CD38 ATM
10 lymphoma 29.6 TP53 MYC IRF4 CD5 CD38 CD19
11 pancytopenia 29.5 TP53 CRP CD5 CD19
12 burkitt lymphoma 29.3 TP53 MYC IRF4 CD19 ATM
13 anemia, autoimmune hemolytic 29.2 ZAP70 IGHV4-38-2 CD5 CD19
14 peripheral t-cell lymphoma 29.2 TP53 NOTCH1 CD5 CD38 CD19
15 myelodysplastic syndrome 29.1 TP53 NOTCH1 MYC CD38 BTK ATM
16 prolymphocytic leukemia 28.9 ZAP70 TP53 MYC CD5 CD38 CD19
17 b-cell lymphoma 28.9 ZAP70 TP53 MYC MIR17 IRF4 CD5
18 marginal zone b-cell lymphoma 28.8 SPN IRF4 IGHV4-38-2 CD5 CD19 BTK
19 diffuse large b-cell lymphoma 28.8 TP53 SPN MYC MIR17 IRF4 CD5
20 mantle cell lymphoma 28.4 TP53 SPN NOTCH1 MYC MIR17 CD5
21 cll/sll 28.4 ZAP70 TP53 SPN IGHV4-38-2 CD5 CD38
22 leukemia, chronic lymphocytic 28.2 ZAP70 TP53 SPN NOTCH1 MYC MIR17
23 waldenstroem's macroglobulinemia 28.2 ZAP70 IRF4 IGHV4-38-2 CD5 CD38 CD19
24 lymphoma, non-hodgkin, familial 27.5 TP53 SPN NOTCH1 MYCN MYC MIR17
25 leukemia, acute myeloid 27.0 TP53 SPN NOTCH1 MYCN MYC MIR17
26 leukemia, acute lymphoblastic 26.9 ZAP70 TP53 NOTCH1 MYCN MYC MIR17
27 kotzot-richter syndrome 11.3
28 bladder lymphoma 10.4 SPN CD5
29 splenic diffuse red pulp small b-cell lymphoma 10.4 TP53 NOTCH1
30 endocrine exophthalmos 10.4 CD5 ATM
31 anal carcinoma in situ 10.4 TP53 MYC
32 lymphoma, hodgkin, classic 10.4
33 lung lymphoma 10.4 SPN CD5
34 leukemia 10.4
35 appendix lymphoma 10.4 MYC CD5
36 maxillary sinus adenocarcinoma 10.3 TP53 ATM
37 primary cutaneous diffuse large b-cell lymphoma, leg type 10.3 MYC IRF4
38 cerebral primitive neuroectodermal tumor 10.3 MYCN MYC
39 suppressor of tumorigenicity 3 10.3 TP53 MYC
40 gastrointestinal lymphoma 10.3 SPN CD5
41 infected hydrocele 10.3 SPN CRP
42 nodular medulloblastoma 10.3 MYCN MYC
43 soft tissue sarcoma 10.3 TP53 CRP
44 lung adenoma 10.3 TP53 NOTCH1 MYC
45 t-cell adult acute lymphocytic leukemia 10.3 TP53 IRF4 CD5
46 breast osteosarcoma 10.3 SPN CD5
47 cervical adenoma malignum 10.3 TP53 ATM
48 breast lymphoma 10.3 SPN IRF4 CD5
49 gallbladder lymphoma 10.2 SPN IRF4 CD5
50 macroglobulinemia 10.2

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 23)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-109 9.75 MYCN
2 Increased shRNA abundance (Z-score > 2) GR00366-A-12 9.75 TP53
3 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.75 CRP NOTCH1 TP53
4 Increased shRNA abundance (Z-score > 2) GR00366-A-132 9.75 TP53
5 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.75 MYCN
6 Increased shRNA abundance (Z-score > 2) GR00366-A-139 9.75 NOTCH1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.75 CRP NOTCH1
8 Increased shRNA abundance (Z-score > 2) GR00366-A-148 9.75 CRP
9 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.75 CRP NOTCH1
10 Increased shRNA abundance (Z-score > 2) GR00366-A-165 9.75 MYCN
11 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.75 TP53
12 Increased shRNA abundance (Z-score > 2) GR00366-A-173 9.75 MYCN
13 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.75 MYCN
14 Increased shRNA abundance (Z-score > 2) GR00366-A-184 9.75 TP53
15 Increased shRNA abundance (Z-score > 2) GR00366-A-19 9.75 TP53
16 Increased shRNA abundance (Z-score > 2) GR00366-A-190 9.75 TP53
17 Increased shRNA abundance (Z-score > 2) GR00366-A-20 9.75 TP53
18 Increased shRNA abundance (Z-score > 2) GR00366-A-28 9.75 NOTCH1
19 Increased shRNA abundance (Z-score > 2) GR00366-A-35 9.75 TP53
20 Increased shRNA abundance (Z-score > 2) GR00366-A-39 9.75 TP53
21 Increased shRNA abundance (Z-score > 2) GR00366-A-6 9.75 NOTCH1
22 Increased shRNA abundance (Z-score > 2) GR00366-A-62 9.75 TP53
23 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.75 CRP

MGI Mouse Phenotypes related to Richter's Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.11 ATM BTK CD19 CD38 IRF4 MLH1
2 hematopoietic system MP:0005397 10.07 ATM BTK CD19 CD38 CD5 IRF4
3 immune system MP:0005387 9.97 ATM BTK CD19 CD38 CD5 CRP
4 digestive/alimentary MP:0005381 9.87 BTK CD19 MLH1 MYC MYCN NOTCH1
5 integument MP:0010771 9.61 ATM BTK CD19 CD5 MLH1 MYC
6 neoplasm MP:0002006 9.28 ATM BTK CD19 IRF4 MLH1 MYC
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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 57)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
ofatumumab Approved Phase 2 679818-59-8 6918251
2
Pembrolizumab Approved Phase 2 1374853-91-4
3
Cytarabine Approved, Investigational Phase 1, Phase 2 147-94-4 6253
4
Fludarabine Approved Phase 1, Phase 2 21679-14-1, 75607-67-9 30751
5
Oxaliplatin Approved, Investigational Phase 1, Phase 2 61825-94-3 5310940 9887054 43805 6857599
6
Vidarabine Approved, Investigational Phase 1, Phase 2 24356-66-9 21704 32326
7
Venetoclax Approved, Investigational Phase 2 1257044-40-8 49846579
8
Zanubrutinib Approved, Investigational Phase 2 1691249-45-2
9
Atezolizumab Approved, Investigational Phase 2 1380723-44-3
10
Blinatumomab Approved, Investigational Phase 2 853426-35-4
11
Lenalidomide Approved Phase 2 191732-72-6 216326
12
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
13
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
14
rituximab Approved Phase 2 174722-31-7 10201696
15
Etoposide Approved Phase 2 33419-42-0 36462
16
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
17
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
18
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
19
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
20
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
21
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
22
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
23
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
24
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
25
Obinutuzumab Approved, Investigational Phase 2 949142-50-1
26
Tyrosine Approved, Investigational, Nutraceutical Phase 1, Phase 2 60-18-4 6057
27
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
28 Anti-Infective Agents Phase 1, Phase 2
29 Antiviral Agents Phase 1, Phase 2
30 Antimetabolites Phase 1, Phase 2
31 Protein Kinase Inhibitors Phase 2
32 Angiogenesis Inhibitors Phase 2
33 Gastrointestinal Agents Phase 2
34 Antiemetics Phase 2
35 BB 1101 Phase 2
36 Antibiotics, Antitubercular Phase 2
37 Etoposide phosphate Phase 2
38 Hormone Antagonists Phase 2
39 glucocorticoids Phase 2
40 Immunosuppressive Agents Phase 2
41 Antirheumatic Agents Phase 2
42 Hormones Phase 2
43 Immunologic Factors Phase 2
44 Immunoglobulins Phase 2
45 Alkylating Agents Phase 2
46 Antineoplastic Agents, Hormonal Phase 2
47 Antibodies Phase 2
48 Antibodies, Monoclonal Phase 2
49 Anti-Bacterial Agents Phase 2
50 Anti-Inflammatory Agents Phase 2

Interventional clinical trials:

(show all 29)
# Name Status NCT ID Phase Drugs
1 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
2 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) Completed NCT02576990 Phase 2
3 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
4 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
5 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
6 A Phase I-II Study of Oxaliplatin, Fludarabine, Cytarabine and Rituximab in Patients With Richter's Transformation, Prolymphocytic Leukemia or Refractory/Relapsed B-Cell Chronic Lymphocytic Leukemia Completed NCT00452374 Phase 1, Phase 2 Cytarabine;Fludarabine;Oxaliplatin;Rituximab
7 BLINAtumomab After R-CHOP Debulking Therapy for Patients With Richter Transformation Recruiting NCT03931642 Phase 2 RCHOP;Blinatumomab
8 Venetoclax Plus Dose-adjusted R-EPOCH or R-CHOP for Richter's Syndrome Recruiting NCT03054896 Phase 2 Venetoclax
9 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
10 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
11 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
12 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
13 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
14 A Phase II Study of Blinatumomab in Richter Transformation Active, not recruiting NCT03121534 Phase 2 Blinatumomab;Dexamethasone
15 Phase I Study of Ublituximab and Umbralisib in Combination With Targeted Immunotherapy in Patients With Relapsed-refractory Chronic Lymphocytic Leukemia (CLL) or Richter's Transformation (RT) of CLL Active, not recruiting NCT02535286 Phase 1, Phase 2 Umbralisib
16 A Phase II Study of MOR00208 in Combination With Lenalidomide for Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia(CLL)/Small Lymphocytic Lymphoma (SLL)/Prolymphocytic Leukemia (PLL), Including Those Who Have Relapsed on Ibrutinib, or Patients With Untreated CLL/SLL/PLL and an Open Pilot Study for Patients With Richters Transformation (RT) Active, not recruiting NCT02005289 Phase 2 lenalidomide
17 Phase II Study of Polatuzumab Vedotin in Combination With Chemotherapy in Subjects With Richter's Transformation Not yet recruiting NCT04679012 Phase 2 Polatuzumab Vedotin;Rituximab;Etoposide;Prednisone;Cyclophosphamide;Hydroxydaunomycin
18 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
19 A Phase II Trial of Early Intervention With Acalabrutinib in Patients With CLL at High Risk for Richter's Transformation Not yet recruiting NCT04660045 Phase 2 Acalabrutinib
20 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
21 A Phase II Study of PNT2258 in Patients With Richter's Transformation (RT) Terminated NCT02378038 Phase 2 PNT2258
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 Obinutuzumab Containing Conditioning Regimen for CLL Patients and Patients With Richter's Transformation Requiring an Allogeneic Stem Cell Transplantation Terminated NCT03153514 Phase 2
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 I Study of Duvelisib in Combination With Nivolumab for Patients With Richter's Syndrome and Transformed Follicular Lymphoma Recruiting NCT03892044 Phase 1 Duvelisib
26 A Phase I Study of PI3Kα,δ Inhibitor Copanlisib in Combination With PD-1 Antagonist Nivolumab in Patients With Transformed Chronic Lymphocytic Leukemia (Richter's Transformation) or Non-Hodgkin Lymphoma Recruiting NCT03884998 Phase 1 Copanlisib
27 Ipilimumab Combined With Ibrutinib and Nivolumab for Patients With Chronic Lymphocytic Leukemia (CLL) and Richter Transformation (RT) Not yet recruiting NCT04781855 Phase 1 Ibrutinib
28 Genomic and Proteomic Study of Richter Syndrome Recruiting NCT03619512
29 Registry of the German CLL Study Group Long Term Follow-up of Patients With CLL, B-PLL, T-PLL, SLL,T or NK-LGL, HCL and Richter's Transformation Recruiting NCT02863692

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
Spleen, Myeloid, Brain, Bone, Bone Marrow, B Cells, T Cells
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Publications for Richter's Syndrome

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Articles related to Richter's Syndrome:

(show top 50) (show all 511)
# Title Authors PMID Year
1
Real-world outcomes following venetoclax therapy in patients with chronic lymphocytic leukemia or Richter syndrome: a FILO study of the French compassionate use cohort. 61
33495922 2021
2
2-[18F]-FDG PET/CT Role in Detecting Richter Transformation of Chronic Lymphocytic Leukemia and Predicting Overall Survival. 61
33386279 2021
3
Synergistic efficacy of dual PI3K-d/g inhibitor Duvelisib with Bcl2 inhibitor Venetoclax in Richter's Syndrome PDX models. 61
33786583 2021
4
Allogeneic hematopoietic cell transplantation is an effective treatment for patients with Richter syndrome: A systematic review and meta-analysis. 61
32473105 2021
5
EBV-positive diffuse large B-cell lymphoma, not otherwise specified masking Richter syndrome. 61
33594655 2021
6
Richter Syndrome. 61
33580422 2021
7
U-RT1 - A new model for Richter transformation. 61
33316538 2021
8
ROR1 targeting with the antibody drug-conjugate VLS-101 is effective in Richter syndrome patient-derived xenograft mouse models. 61
33512452 2021
9
HELQ and EGR3 expression correlate with IGHV mutation status and prognosis in chronic lymphocytic leukemia. 61
33485349 2021
10
Angioimmunoblastic T-Cell Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma: A Novel Form of Composite Lymphoma Potentially Mimicking Richter Syndrome. 61
33739791 2021
11
Richter's Syndrome of the Central Nervous System. 61
33397524 2021
12
Lymphoid enhancer binding factor 1 (LEF1) expression is significantly higher in Hodgkin lymphoma associated with Richter syndrome relative to de novo classic Hodgkin lymphoma. 61
32977233 2020
13
Genomic and transcriptomic correlates of Richter's transformation in Chronic Lymphocytic Leukemia. 61
33206936 2020
14
Cutaneous manifestations of B-cell chronic lymphocytic leukemia. 61
32889697 2020
15
Diagnosis and Treatment of Chronic Lymphocytic Leukemia: Recommendations of the French CLL Study Group (FILO). 61
33062946 2020
16
Clinical activity of axicabtagene ciloleucel in adult patients with Richter syndrome. 61
33002129 2020
17
Visual Dermatology: Sustained Remission of Richter's Syndrome. 61
33023345 2020
18
Prognostication in Richter syndrome: context is everything. 61
32420622 2020
19
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
20
Ulcerated cutaneous Richter syndrome. 61
33054942 2020
21
[Clinical value of PET/CT in the diagnosis of Richter syndrome]. 61
32942827 2020
22
Pembrolizumab in relapsed or refractory Richter syndrome. 61
32544980 2020
23
Prognostic Significance of PET/CT in Patients with Chronic Lymphocytic Leukemia (CLL) Treated with Frontline Chemoimmunotherapy. 61
32635175 2020
24
Correction: Bidirectional linkage between the B-cell receptor and NOTCH1 in chronic lymphocytic leukemia and in Richter's syndrome: therapeutic implications. 61
31836851 2020
25
ROR1 Is Expressed in Diffuse Large B-Cell Lymphoma (DLBCL) and a Small Molecule Inhibitor of ROR1 (KAN0441571C) Induced Apoptosis of Lymphoma Cells. 61
32586008 2020
26
Multiple B cell malignancies in patients with chronic lymphocytic leukemia: epidemiology, pathology, and clinical implications. 61
31928278 2020
27
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
28
Duodenal stricture secondary to Richter's syndrome. 61
31590994 2020
29
Richter's syndrome of T-cell lineage (T rex lymphoma). 61
31910677 2020
30
A case of T-cell chronic lymphocytic leukemia progressing to Richter syndrome with central nervous system involvement in a dog. 61
32215932 2020
31
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
32
Bidirectional linkage between the B-cell receptor and NOTCH1 in chronic lymphocytic leukemia and in Richter's syndrome: therapeutic implications. 61
31467429 2020
33
Prognostic Value of Baseline Total Metabolic Tumor Volume Measured on FDG PET in Patients With Richter Syndrome. 61
31876819 2020
34
Microenvironment Remodeling and Subsequent Clinical Implications in Diffuse Large B-Cell Histologic Variant of Richter Syndrome. 61
33381116 2020
35
[Sequential development of mantle cell lymphoma following chronic lymphocytic leukemia]. 61
32224586 2020
36
Genetic Loss of LCK Kinase Leads to Acceleration of Chronic Lymphocytic Leukemia. 61
32983140 2020
37
The Occurrence of Richter's Syndrome during Treatment with Obinutuzumab and Chlorambucil. 61
32724682 2020
38
Computed tomography textural analysis for the differentiation of chronic lymphocytic leukemia and diffuse large B cell lymphoma of Richter syndrome. 61
31236702 2019
39
Transformation of monoclonal B lymphocytosis to Epstein-Barr virus-positive large B-cell lymphoma with intermediate features between diffuse large B-cell lymphoma and classic Hodgkin lymphoma. 61
33437870 2019
40
Rare case of Richter syndrome with testicular involvement successfully obtained good prognosis with rapid operation and immunochemotherapy. 61
32743423 2019
41
High-grade B-Cell lymphoma with MYC and BCL6 rearrangements associated with Richter transformation of chronic lymphocytic leukemia. 61
31440479 2019
42
Richter syndrome epidemiology in a large population based chronic lymphocytic leukemia cohort from Norway. 61
30986631 2019
43
The clinical study on treatment of CD19-directed chimeric antigen receptor-modified T cells in a case of refractory Richter syndrome. 61
31050207 2019
44
Durable Response to Venetoclax Monotherapy in Richter's Syndrome: A Case Report and Review of Literature. 61
32300445 2019
45
The involvement of microRNA in the pathogenesis of Richter syndrome. 61
30409799 2019
46
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
47
BCR kinase inhibitors, idelalisib and ibrutinib, are active and effective in Richter syndrome. 61
29974955 2019
48
Use of PD-1 (PDCD1) inhibitors for the treatment of Richter syndrome: experience at a single academic centre. 61
30028000 2019
49
Loss of NFAT2 expression results in the acceleration of clonal evolution in chronic lymphocytic leukemia. 61
30556925 2019
50
Richter Syndrome Presenting With Colon Localization. 61
30516680 2019
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Variations for Richter's Syndrome

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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 22)
# Super pathways Score Top Affiliating Genes
1
PI3K-Akt signaling pathway 36
Show member pathways
 12.83 TP53 NOTCH1 MYC CD19 ATM
2
TCR Signaling (Qiagen) 63
Show member pathways
 12.75 ZAP70 TP53 MLH1 BTK ATM
3
NF-kappaB Signaling 4
12.32 ZAP70 IRF4 CD38 CD19 BTK
4
B cell receptor signaling pathway (KEGG) 36
Show member pathways
 12.24 MYC IRF4 CD19 BTK
5
DNA Damage Response 1
Show member pathways
 12.2 TP53 MYC MIR17 ATM
6
Immune response IFN alpha/beta signaling pathway 23
Show member pathways
 12.12 TP53 MYC IRF4 ATM
7
MicroRNAs in cancer 36
12.12 TP53 NOTCH1 MYC MIR181B1 MIR17 ATM
8
Transcriptional misregulation in cancer 36
12.09 TP53 MYCN MYC ATM
9
DNA Damage Response (only ATM dependent) 1
11.83 TP53 MYC ATM
10
Interleukin-4 and 13 signaling 65
11.82 TP53 MYC IRF4
11
NF-kappa B signaling pathway 36
11.76 ZAP70 BTK ATM
12
Hematopoietic cell lineage 36
11.73 CD5 CD38 CD19
13
Platinum drug resistance 36
11.58 TP53 MLH1 ATM
14
Dendritic Cells Developmental Lineage Pathway 64
11.49 SPN IRF4 CD38 CD19
15
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 64
11.42 SPN NOTCH1 CD5 CD38 CD19
16
Integrated Cancer Pathway 1
11.32 TP53 MYC ATM
17
DNA damage_ATM/ATR regulation of G1/S checkpoint 23
11.28 TP53 MYC ATM
18
miRNAs involved in DNA damage response 1
11.26 TP53 MYC MIR181B1 ATM
19
Primary immunodeficiency 36
11.19 ZAP70 CD19 BTK
20
TP53 Network 1
Show member pathways
 10.89 TP53 MYC ATM
21
Pathways Affected in Adenoid Cystic Carcinoma 1
10.84 TP53 NOTCH1 MYCN MYC ATM
22
Notch Pathway 69
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 14)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 10.01 TP53 NOTCH1 MYCN MYC IRF4 ATM
2 negative regulation of gene expression GO:0010629 9.8 TP53 NOTCH1 MYCN MIR17
3 positive regulation of transcription, DNA-templated GO:0045893 9.73 TP53 NOTCH1 MYCN MYC IRF4 CD38
4 apoptotic signaling pathway GO:0097190 9.58 CD5 CD38 BTK
5 negative regulation of toll-like receptor signaling pathway GO:0034122 9.52 MIR17 IRF4
6 replicative senescence GO:0090399 9.51 TP53 ATM
7 positive regulation of response to DNA damage stimulus GO:2001022 9.48 MYC ATM
8 cardiac septum morphogenesis GO:0060411 9.46 TP53 NOTCH1
9 cell cycle arrest GO:0007050 9.46 TP53 NOTCH1 MYC ATM
10 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.43 TP53 MYCN
11 meiotic telomere clustering GO:0045141 9.37 MLH1 ATM
12 positive regulation of transcription from RNA polymerase II promoter in response to hypoxia GO:0061419 9.32 TP53 NOTCH1
13 B cell receptor signaling pathway GO:0050853 9.26 IGHV4-38-2 CD38 CD19 BTK
14 positive regulation of gene expression GO:0010628 9.1 TP53 NOTCH1 MYCN MYC CRP ATM
Sources

Sources for Richter's Syndrome

About this section
3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 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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