Richter's Syndrome disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

MCID: RCH001 MIFTS: 45	Richter's Syndrome Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases	Data Licensing For inquiries, contact: [email protected]
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Aliases & Classifications for Richter's Syndrome

MalaCards integrated aliases for Richter's Syndrome:

Name: Richter's Syndrome ¹¹ ¹⁴ ⁷¹

Richter Syndrome ¹¹ ¹⁹ ⁷⁵

Richter Transformation ¹⁹

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Immune diseases Blood diseases

See all MalaCards categories (disease lists)

ICD10: ³¹

Neoplasms

Malignant neoplasms, stated or presumed to be primary, of lymphoid, haematopoietic and related tissue

Lymphoid leukaemia

Chronic lymphocytic leukaemia of B-cell type

External Ids:

Disease Ontology ¹¹ DOID:1703

NCIt ⁴⁹ C35424

SNOMED-CT ⁶⁸ 277550009

ICD10 ³¹ C91.1

UMLS ⁷¹ C0349631

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Summaries for Richter's Syndrome

GARD: ¹⁹ 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 leukemia, chronic lymphocytic 2 and macroglobulinemia. An important gene associated with Richter's Syndrome is IGHV4-38-2 (Immunoglobulin Heavy Variable 4-38-2), and among its related pathways/superpathways are Innate Immune System and ERK Signaling. The drugs Nivolumab and Ofatumumab have been mentioned in the context of this disorder. Affiliated tissues include spleen, liver and t cells, and related phenotypes are Reduced mammosphere formation and cellular

Wikipedia: ⁷⁵ Richter's transformation (RT), also known as Richter's syndrome, is the conversion of chronic... more...

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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 298)

#	Related Disease	Score	Top Affiliating Genes
1	leukemia, chronic lymphocytic 2	30.8	ZAP70 CD5 CD38 BCL2 ATM
2	macroglobulinemia	30.2	TP53 PAX5 BTK BCL2
3	immunodeficiency with hyper-igm, type 1	30.2	IGHV4-38-2 CD19 BTK
4	intravascular large b-cell lymphoma	30.1	BCL6 BCL2
5	skin carcinoma	30.0	TP53 NOTCH1 MYC BCL2
6	lymphoproliferative syndrome	29.8	TNFRSF8 TCL1A CD5 BCL6 BCL2
7	reticulosarcoma	29.8	TNFRSF8 PAX5 MYC BCL6 BCL2
8	lymphoma aids related	29.8	TCL1A MYC BCL6
9	pleural empyema	29.8	TNFRSF8 CD5 BCL6
10	anemia, autoimmune hemolytic	29.7	ZAP70 IGHV4-38-2 CD5 CD38 CD19 BTK
11	lymphoma, hodgkin, classic	29.7	ZAP70 TP53 TNFRSF8 PAX5 BCL6 BCL2
12	lymphosarcoma	29.7	TNFRSF8 BCL6 BCL2
13	myelodysplastic syndrome	29.6	TP53 MYC CD38 CD19 BCL2
14	chromosome 13q14 deletion syndrome	29.6	ZAP70 TP53 IGHV4-38-2 CD5 CD38 ATM
15	deficiency anemia	29.6	TP53 CD5 CD38 CD19 ATM
16	pancytopenia	29.5	TP53 CD5 CD22 CD19
17	b-cell prolymphocytic leukemia	29.5	IGHV4-38-2 CD79B CD5 CD22 CD19
18	adult t-cell leukemia/lymphoma	29.4	TP53 TNFRSF8 MYC CD5 BCL2
19	gastric lymphoma	29.2	TNFRSF8 IGHV4-38-2 CD5 BIRC3 BCL6 BCL2
20	angioimmunoblastic t-cell lymphoma	29.2	TNFRSF8 PAX5 IGHV4-38-2 CD5 BCL6
21	leukemia	29.2	ZAP70 TP53 TCL1B TCL1A NOTCH1 MYC
22	prolymphocytic leukemia	29.2	ZAP70 TP53 TCL1B TCL1A MYC CD5
23	bone lymphoma	29.1	TNFRSF8 PAX5 CD5 BCL6 BCL2
24	hematologic cancer	29.1	ZAP70 TNFRSF8 PAX5 NOTCH1 BCL2 ATM
25	diffuse large b-cell lymphoma	29.1	TNFRSF8 PAX5 MYC IGHV4-38-2 CD79B CD5
26	plasma cell neoplasm	29.1	TP53 PAX5 MYC IGHV4-38-2 CD38 CD19
27	t-cell prolymphocytic leukemia	29.1	TP53 TNFRSF8 TCL1B TCL1A IGHV4-38-2 CD5
28	waldenstroem's macroglobulinemia	29.0	ZAP70 PAX5 IGHV4-38-2 CD79B CD5 CD38
29	immune deficiency disease	29.0	ZAP70 TNFRSF8 CD5 CD38 BTK BCL6
30	follicular lymphoma	28.9	TP53 TCL1A PAX5 MYC CD5 BIRC3
31	plasmablastic lymphoma	28.9	TNFRSF8 PAX5 MYC IGHV4-38-2 CD38 BCL6
32	lymphatic system disease	28.8	TP53 TNFRSF8 MYC IGHV4-38-2 CD5 CD19
33	t-cell acute lymphoblastic leukemia	28.6	ZAP70 TNFRSF8 TCL1B TCL1A NOTCH1 MYC
34	burkitt lymphoma	28.6	TP53 TNFRSF8 TCL1A PAX5 MYC CD19
35	peripheral t-cell lymphoma	28.5	ZAP70 TP53 TNFRSF8 TCL1A PAX5 CD5
36	mature t-cell and nk-cell lymphoma	28.5	TP53 TNFRSF8 PAX5 MYC IGHV4-38-2 CD5
37	composite lymphoma	28.3	TNFRSF8 PAX5 IGHV4-38-2 CD5 CD22 BCL6
38	central nervous system lymphoma	28.3	TNFRSF8 PAX5 MYC IGHV4-38-2 CD79B CD5
39	lymphoma	28.3	TP53 TNFRSF8 TCL1B TCL1A PAX5 MYC
40	chronic lymphocytic leukemia/small lymphocytic lymphoma	27.8	ZAP70 TP53 TNFRSF8 PAX5 IGHV4-38-2 CD79B
41	b-cell lymphoma	27.7	ZAP70 TP53 TNFRSF8 TCL1A PAX5 MYC
42	leukemia, acute lymphoblastic	27.4	ZAP70 TP53 TCL1A PAX5 NOTCH1 MYC
43	marginal zone b-cell lymphoma	27.1	TNFRSF8 PAX5 MYC IGHV4-38-2 CD79B CD5
44	leukemia, chronic lymphocytic	27.0	ZAP70 TP53 TNFRSF8 TCL1B TCL1A RPS15
45	lymphoma, non-hodgkin, familial	26.9	TP53 TNFRSF8 TCL1A PAX5 NOTCH1 MYC
46	leukemia, acute myeloid	26.9	TP53 TNFRSF8 PAX5 NOTCH1 MYC IGHV4-38-2
47	mantle cell lymphoma	26.1	ZAP70 TP53 TNFRSF8 TCL1B TCL1A PAX5
48	kotzot-richter syndrome	11.2
49	solitary osseous plasmacytoma	10.3	IGHV4-38-2 CD38
50	congenital hypogammaglobulinemia	10.3	CD19 BTK

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

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Symptoms & Phenotypes for Richter's Syndrome

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

²⁵

#	Description	GenomeRNAi Source Accession	Score	Top Affiliating Genes
1	Reduced mammosphere formation	GR00396-S	9.17	ATM BCL6 CD22 CD5 MYC NOTCH1

MGI Mouse Phenotypes related to Richter's Syndrome:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	cellular	MP:0005384	10.07	ATM BCL2 BCL6 BIRC3 BTK CD19
2	immune system	MP:0005387	10.06	ATM BCL2 BCL6 BIRC3 BTK CD19
3	normal	MP:0002873	10.02	BCL6 BIRC3 CD19 CD5 CD79B MYC
4	neoplasm	MP:0002006	9.97	ATM BCL2 BTK CD19 MYC NOTCH1
5	hematopoietic system	MP:0005397	9.86	ATM BCL2 BCL6 BIRC3 BTK CD19
6	integument	MP:0010771	9.32	ATM BCL2 BCL6 BTK CD19 CD5

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Drugs & Therapeutics for Richter's Syndrome

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

(show top 50) (show all 90)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Nivolumab

Approved

Phase 2

946414-94-4

Ofatumumab

Approved

Phase 2

679818-59-8

Dexamethasone acetate

Approved, Investigational, Vet_approved

Phase 2

1177-87-3

3680

Dexamethasone

Approved, Investigational, Vet_approved

Phase 2

50-02-2

3003 5743

Lenalidomide

Approved

Phase 1, Phase 2

191732-72-6

216326

Atezolizumab

Approved, Investigational

Phase 2

1380723-44-3

Durvalumab

Approved, Investigational

Phase 2

1428935-60-7

Zanubrutinib

Approved, Investigational

Phase 2

1691249-45-2

135565884

Fludarabine

Approved

Phase 2

75607-67-9, 21679-14-1

30751 657237

Pembrolizumab

Approved

Phase 1, Phase 2

1374853-91-4

254741536

Idelalisib

Approved

Phase 2

870281-82-6

11654566 11625818

Blinatumomab

Approved, Investigational

Phase 2

853426-35-4

Rituximab

Approved

Phase 2

174722-31-7

Prednisolone phosphate

Approved, Vet_approved

Phase 2

302-25-0

Prednisolone acetate

Approved, Vet_approved

Phase 2

52-21-1

Prednisolone

Approved, Vet_approved

Phase 2

50-24-8

4894 5755

Methylprednisolone hemisuccinate

Approved

Phase 2

2921-57-5

1875

Methylprednisolone

Approved, Vet_approved

Phase 2

83-43-2

4159 6741

Venetoclax

Approved, Investigational

Phase 2

1257044-40-8

49846579

Etoposide

Approved

Phase 2

33419-42-0

36462

Valaciclovir

Approved, Investigational

Phase 2

124832-27-5, 124832-26-4

60773 135398742

Allopurinol

Approved

Phase 2

315-30-0

2094 135401907

Coal tar

Approved

Phase 2

8007-45-2

Acalabrutinib

Approved, Investigational

Phase 1, Phase 2

1420477-60-6

71226663 71226662

Cyclophosphamide

Approved, Investigational

Phase 2

50-18-0, 6055-19-2

2907

Vincristine

Approved, Investigational

Phase 2

2068-78-2, 57-22-7

5978

Doxorubicin

Approved, Investigational

Phase 2

23214-92-8

31703

Obinutuzumab

Approved, Investigational

Phase 2

949142-50-1

Prednisone

Approved, Vet_approved

Phase 2

53-03-2

5865

Gemcitabine

Approved

Phase 1, Phase 2

95058-81-4, 122111-03-9

60750

Sirolimus

Approved, Investigational

Phase 1, Phase 2

53123-88-9

5284616 6436030

Busulfan

Approved, Investigational

Phase 1, Phase 2

55-98-1

2478

Lenograstim

Approved, Investigational

Phase 1, Phase 2

135968-09-1

Levoleucovorin

Approved, Experimental, Investigational

Phase 1, Phase 2

68538-85-2, 58-05-9, 73951-54-9

149436 6006

Methotrexate

Approved

Phase 1, Phase 2

1959-05-2, 59-05-2

4112 126941

Tacrolimus

Approved, Investigational

Phase 1, Phase 2

104987-11-3

6473866 445643

Clofarabine

Approved, Investigational

Phase 1, Phase 2

123318-82-1

119182

D-Tyrosine

Approved, Experimental, Investigational, Nutraceutical

Phase 1, Phase 2

133585-56-5, 60-18-4, 556-02-5

1153 6057

Folic acid

Approved, Nutraceutical, Vet_approved

Phase 1, Phase 2

59-30-3

6037

Prednisolone hemisuccinate

Experimental

Phase 2

2920-86-7

4897

Immune Checkpoint Inhibitors

Phase 2

Antiemetics

Phase 2

BB 1101

Phase 2

Gastrointestinal Agents

Phase 2

Antibodies, Monoclonal

Phase 2

Muromonab-CD3

Phase 1, Phase 2

Angiogenesis Inhibitors

Phase 1, Phase 2

Immunoglobulins, Intravenous

Phase 1, Phase 2

Immunoconjugates

Phase 1, Phase 2

Immunoglobulin G

Phase 2

Interventional clinical trials:

(show all 46)

#	Name	Status	NCT ID	Phase	Drugs
1	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
2	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
3	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
4	A Phase I/II Study of Syk Inhibitor Entospletinib (GS-9973) in Combination With Obinutuzumab in Patients With Relapsed/Refractory Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) and B-Cell Malignancies	Completed	NCT03010358	Phase 1, Phase 2	Entospletinib
5	Nivolumab Combined With Ibrutinib for Relapsed, Refractory or High-Risk Untreated Patients With Chronic Lymphocytic Leukemia (CLL)	Completed	NCT02420912	Phase 2	Ibrutinib
6	Trial of Immune Reconstitution With Activated T-Cells in Patients With Chronic Lymphocytic Leukemia (CLL)	Completed	NCT02530515	Phase 2
7	A Phase II Study of Blinatumomab in Richter Transformation	Completed	NCT03121534	Phase 2	Blinatumomab;Dexamethasone
8	A Phase 1b/2, Open-Label, Safety and Efficacy Study of Epcoritamab (GEN3013; DuoBody®-CD3 X CD20) in Relapsed/Refractory Chronic Lymphocytic Leukemia and Richter's Syndrome	Recruiting	NCT04623541	Phase 1, Phase 2
9	Venetoclax Plus Dose-adjusted R-EPOCH or R-CHOP for Richter's Syndrome	Recruiting	NCT03054896	Phase 2	Venetoclax
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	A Prospective, Phase-II Study to Evaluate the Efficacy and Safety of Obinutuzumab, Ibrutinib, and Venetoclax in Patients With Richter's Syndrome. GIVeRS Protocol: On Behalf of the Israeli CLL Study Group	Recruiting	NCT04939363	Phase 2
12	Phase II Study of Polatuzumab Vedotin in Combination With Chemotherapy in Subjects With Richter's Transformation	Recruiting	NCT04679012	Phase 2	Polatuzumab Vedotin;Rituximab;Etoposide;Prednisone;Cyclophosphamide;Hydroxydaunomycin
13	Safety and Tolerability of Myeloablative Conditioning and Autologous Stem Cell Transplantation Followed by Polatuzumab Vedotin (PV) Immunoconjugate Therapy in Patients With B-cell Non-Hodgkin and Hodgkin Lymphoma	Recruiting	NCT04491370	Phase 1, Phase 2	Polatuzumab vedotin
14	A Phase I/II Open Label, Single Center, Study of the Combination of ALX148, Rituximab and Lenalidomide in Patients With Indolent and Aggressive B-Cell Non-Hodgkin Lymphoma	Recruiting	NCT05025800	Phase 1, Phase 2	CD47 Antagonist ALX148;Lenalidomide
15	Phase II Study of a Combination Therapy of Acalabrutinib, Venetoclax and Durvalumab (MEDI4736) in Patients With Richter Transformation From Chronic Lymphocytic Leukemia (CLL)	Recruiting	NCT05388006	Phase 2	Acalabrutinib;Venetoclax
16	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
17	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
18	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
19	A Phase 2, Open-Label, Multicenter, Basket Study Evaluating the Safety and Efficacy of Brexucabtagene Autoleucel in Adults With Rare B-cell Malignancies (ZUMA-25)	Recruiting	NCT05537766	Phase 2	Cyclophosphamide;Fludarabine
20	A Multicenter, Open-label, Phase 2 Basket Study to Evaluate the Safety and Efficacy of MK-2140 as a Monotherapy and in Combination in Participants With Aggressive and Indolent B-cell Malignancies	Recruiting	NCT05458297	Phase 2	Nemtabrutinib
21	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
22	A Phase 1b/2 Proof-of-Concept Study of the Combination of ACP-196 (Acalabrutinib) and Pembrolizumab in Subjects With Hematologic Malignancies	Active, not recruiting	NCT02362035	Phase 1, Phase 2	Acalabrutinib;Pembrolizumab
23	A Phase II Study of Anti-PD-1 Antibody (MK-3475) in Relapsed/Refractory Chronic Lymphocytic Leukemia (CLL) and Other Low Grade B Cell Non-Hodgkin Lymphoma (NHL)	Active, not recruiting	NCT02332980	Phase 2	Ibrutinib;Idelalisib
24	BLINAtumomab After R-CHOP Debulking Therapy for Patients With Richter Transformation	Active, not recruiting	NCT03931642	Phase 2	RCHOP;Blinatumomab
25	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
26	Time-limited Triplet Combination of Pirtobrutinib, Venetoclax, and Obinutuzumab for Patients With Treatment-naïve Chronic Lymphocytic Leukemia (CLL) or Richter Transformation (RT)	Not yet recruiting	NCT05536349	Phase 2	Pirtobrutinib;Obinutuzumab;Venetoclax;Valacyclovir;Allopurinol
27	A Multicenter Phase 2 Study of R-EPOCH in Combination With Ibrutinib for Patients With Classical Richter Transformation of Chronic Lymphocytic Leukemia	Not yet recruiting	NCT04992377	Phase 2	R-EPOCH in Combination With Ibrutinib
28	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
29	A Phase 1/2 Proof-of-Concept Study of the Combination of Acalabrutinib and Vistusertib in Subjects With Relapsed/Refractory B-cell Malignancies	Terminated	NCT03205046	Phase 1, Phase 2	acalabrutinib;vistusertib
30	Clofarabine, Gemcitabine, and Busulfan Followed by Allogeneic Stem Cell Transplantation for Chronic Lymphocytic Leukemia (CLL)	Terminated	NCT01629511	Phase 1, Phase 2	Busulfan;Clofarabine;Gemcitabine;Methotrexate;Tacrolimus
31	A Phase 2 Feasibility Study of Sotrastaurin for Relapsed and Refractory CLL/SLL/PLL/RT	Withdrawn	NCT02285244	Phase 2	sotrastaurin acetate
32	Obinutuzumab, High Dose Methylprednisolone (HDMP), and Lenalidomide for the Treatment of Patients With Richter's Syndrome	Completed	NCT03113695	Phase 1	Obinutuzumab;lenalidomide;HDMP
33	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	Completed	NCT03263637	Phase 1	AZD4573
34	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	Completed	NCT02535286	Phase 1	Umbralisib
35	A Phase 1 Study of Lenalidomide Maintenance Following Allogeneic Hematopoietic Cell Transplantation in Patients With Select High Risk Hematological Malignancies	Completed	NCT01254578	Phase 1	Lenalidomide
36	An Open-label, Multicenter Phase 1 Dose Escalation Study to Characterize Safety, Tolerability, Preliminary Antitumor Activity, Pharmacokinetics, and Maximum Tolerated Dose of VIP152 in Subjects With Relapsed/Refractory Chronic Lymphocytic Leukemia or Richter Syndrome	Recruiting	NCT04978779	Phase 1	VIP152
37	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
38	Ipilimumab Combined With Ibrutinib and Nivolumab for Patients With Chronic Lymphocytic Leukemia (CLL) and Richter Transformation (RT)	Recruiting	NCT04781855	Phase 1	Ibrutinib
39	A Multicenter, Open-label, Phase 1 Study Evaluating the Safety and Tolerability of HMPL-760 in Patients With Previously Treated Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) or Other Non-Hodgkin Lymphoma (NHL)	Recruiting	NCT05176691	Phase 1	HMPL-760
40	A Phase 1/1b Study Evaluating the Safety, Pharmacokinetics, and Preliminary Efficacy of LP-118 in Subjects With Relapsed or Refractory Hematological Malignancies	Recruiting	NCT04771572	Phase 1	LP-118
41	A Phase 1a, Dose Escalation, Safety and Tolerability Study of NX-1607, a Casitas B-lineage Lymphoma Proto-oncogene (CBL-B) Inhibitor, in Adults With Advanced Malignancies, With Phase 1b Expansion in Select Tumor Types	Recruiting	NCT05107674	Phase 1	NX-1607
42	A Phase I Study of Duvelisib in Combination With Nivolumab for Patients With Richter's Syndrome and Transformed Follicular Lymphoma	Active, not recruiting	NCT03892044	Phase 1	Duvelisib
43	A Pilot Phase I Study of Atezolizumab (MPDL3280A) in Combination With Immunogenic Chemotherapy (Gemcitabine-Oxaliplatin) and Rituximab for Transformed Diffuse Large B-Cell Lymphoma	Active, not recruiting	NCT03321643	Phase 1	Atezolizumab;Gemcitabine;Oxaliplatin
44	A Phase I Study of Pevonedistat (MLN4924, TAK924) in Combination With Ibrutinib in Patients With Relapsed/Refractory Chronic Lymphocytic Leukemia and Non-Hodgkin Lymphoma	Active, not recruiting	NCT03479268	Phase 1	Ibrutinib;Pevonedistat
45	A Phase 1 Dose-Escalation and Cohort-Expansion Study of VLS-101 in Subjects With Hematological Malignancies (waveLINE-001)	Active, not recruiting	NCT03833180	Phase 1	Zilovertamab vedotin
46	Genomic and Proteomic Study of Richter Syndrome	Unknown status	NCT03619512

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

Organs/tissues related to Richter's Syndrome:

MalaCards : Spleen, Liver, T Cells, Bone Marrow, B Cells, Lymph Node, Bone

Sources

Publications for Richter's Syndrome

Articles related to Richter's Syndrome:

(show top 50) (show all 728)

#	Title	Authors	PMID	Year
1	Necrotizing herpes simplex lymphadenitis in a patient with chronic lymphocytic leukaemia/small lymphocytic lymphoma, clinically masquerading as Richter transformation. ⁶²	Carlsen ED...Aggarwal N	35811089	2022
2	Long-term follow-up and future direction on the management of chronic lymphocytic leukemia/small lymphocytic leukemia. ⁶²	Hanna KS...Ojulu A	35635742	2022
3	Macrophage- and BCR-derived but not TLR-derived signals support the growth of CLL and Richter syndrome murine models in vivo. ⁶²	Martines C...Efremov DG	36084319	2022
4	In vivo modeling of CLL transformation to Richter's syndrome reveals convergent evolutionary paths and therapeutic vulnerabilities. ⁶²	Ten Hacken E...Wu CJ	36468984	2022
5	Minimal residual disease-driven treatment intensification with sequential addition of ibrutinib to venetoclax in R/R CLL. ⁶²	Scarfo L...Ghia P	35921541	2022
6	Mitochondrial DNA mutations as natural barcodes for lineage tracing of murine tumor models. ⁶²	Penter L...Wu CJ	36469010	2022
7	Richter transformation in Chronic Lymphocytic Leukemia. ⁶²	Innocenti I...Laurenti L	36433773	2022
8	Insights from a patient with chronic lymphocytic leukemia complicating ALK+ anaplastic large cell lymphoma. ⁶²	Lin W...Asakawa T	36457588	2022
9	Deregulation and epigenetic modification of BCL2-family genes cause resistance to venetoclax in hematologic malignancies. ⁶²	Thomalla D...Frenzel LP	35704690	2022
10	An experience with ibrutinib monotherapy for Richter's syndrome isolated in the central nervous system. ⁶²	Nato Y...Miyashita H	36436931	2022
11	TP53/PLCG2-mutated diffuse large B-cell lymphoma richter transformation (DLBCL-RT) of CLL with unusual CD2 and PD-1 expression. ⁶²	Symes E...Venkataraman G	35719104	2022
12	Richter transformation to aggressive plasmablastic neoplasm related to selection of a BTK-mutated clone in a patient with CLL/SLL treated by ibrutinib. ⁶²	Aqil B...Chen Q	36336991	2022
13	A Phase 2 Study of Nivolumab Combined with Ibrutinib in Patients with Diffuse Large B-cell Richter Transformation of CLL. ⁶²	Jain N...Wierda WG	36287248	2022
14	Early Clones Expand in Richter Transformation of Chronic Lymphocytic Leukemia. ⁶²		35984230	2022
15	Long-term effect of venetoclax therapy in a patient with the transformation of chronic lymphocytic leukemia into Richter's syndrome. ⁶²	Vladimirovna TV...Anatolievich RO	36299674	2022
16	Hodgkin's variant of Richter transformation during ibrutinib therapy: A case report and review of the literature. ⁶²	Afonso C...Carda JP	36225619	2022
17	Richter Syndrome: From Molecular Pathogenesis to Druggable Targets. ⁶²	Mouhssine S...Gaidano G	36230566	2022
18	Emerging Therapies for the Management of Richter Transformation. ⁶²	Smyth E...Cheah CY	36130148	2022
19	Distinct immune-response profile of Richter transformation chronic lymphocytic leukemia (CLL), defined by high expression of PD1, LAG3, TIM3 and IL-10. ⁶²	Behdad A...Chen QC	36102789	2022
20	Clonally unrelated Richter syndrome are truly de novo diffuse large B-cell lymphomas with a mutational profile reminiscent of clonally related Richter syndrome. ⁶²	Favini C...Moia R	35829664	2022
21	Clinical outcomes in patients with chronic lymphocytic leukemia with disease progression on ibrutinib. ⁶²	Hampel PJ...Parikh SA	36050317	2022
22	Anti-CD37 α-amanitin-conjugated antibodies as potential therapeutic weapons for Richter syndrome. ⁶²	Vaisitti T...Deaglio S	35914223	2022
23	A phase two study of high dose blinatumomab in Richter's syndrome. ⁶²	Thompson PA...Wierda WG	35941212	2022
24	The clonal evolution of Richter transformation cells uncovers therapeutic vulnerabilities. ⁶²		35953722	2022
25	Richter's syndrome in central nervous system with MYD88L265P and CD79b mutation responded well to ibrutinib containing chemotherapy: a case report and review of the literature. ⁶²	Wang W...Zhou D	35484253	2022
26	Detection of early seeding of Richter transformation in chronic lymphocytic leukemia. ⁶²	Nadeu F...Campo E	35953718	2022
27	Outcomes of Hodgkin variant Richter transformation in chronic lymphocytic leukaemia and small lymphocytic lymphoma in British Columbia. ⁶²	Zhu K...Gerrie AS	35567407	2022
28	Hodgkin Lymphoma and Hairy Cell Leukemia Arising from Chronic Lymphocytic Leukemia: Case Reports and Literature Review. ⁶²	D'Addona M...Selleri C	36012912	2022
29	Artificial intelligence-assisted mapping of proliferation centers allows the distinction of accelerated phase from large cell transformation in chronic lymphocytic leukemia. ⁶²	El Hussein S...Khoury JD	35132162	2022
30	Unusual Illustration of Richter Transformation in Chronic Lymphocytic Leukemia on FDG PET/CT. ⁶²	Liu Y	35307722	2022
31	Selinexor Combined with Ibrutinib Demonstrates Tolerability and Safety in Advanced B-Cell Malignancies: A Phase I Study. ⁶²	Stephens DM...Woyach JA	35608822	2022
32	The Pt(S-pr-thiosal)2 and BCL1 Leukemia Lymphoma: Antitumor Activity In Vitro and In Vivo. ⁶²	Silconi ZB...Milovanovic J	35897737	2022
33	Aberrant expression of lymphoid enhancer-binding factor 1 in Hodgkin lymphoma. ⁶²	Ravindran A...Shi M	35421421	2022
34	Richter Transformation: Clinical Manifestations, Evaluation, and Management. ⁶²	Douglas M	35910504	2022
35	SOHO State of the Art Updates and Next Questions \| Management of Most Difficult Cases of Chronic Lymphocytic Leukemia: Relapse After Both BTK and BCL2 Inhibition and Richter Transformation. ⁶²	Burke JM	35577753	2022
36	Dual peaks on flow cytometric DNA ploidy analysis in chronic lymphocytic leukemia with Richter transformation. ⁶²	Devi Sundaresan D...Malhotra P	35560867	2022
37	An Approach to Diagnosis of Richter Transformation in Chronic Lymphocytic Leukemia. ⁶²	Broadway-Duren J	35910501	2022
38	Distinct Chemokine Receptor Expression Profiles in De Novo DLBCL, Transformed Follicular Lymphoma, Richter's Trans-Formed DLBCL and Germinal Center B-Cells. ⁶²	Uhl B...Deutsch AJ	35887224	2022
39	[Chronic lymphocytic leukemia]. ⁶²	Gauthier M	35184869	2022
40	Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma With Secondary Acquisition of t(11;14)(q13;q32)/CCND1-IGH: A Rare Variant Of Richter Transformation to Mantle Cell Lymphoma. ⁶²	Zhao Y...Wang E	34840090	2022
41	Chronic Lymphocytic Leukemia Progression Diagnosis with Intrinsic Cellular Patterns via Unsupervised Clustering. ⁶²	Chen P...Wu J	35626003	2022
42	Infection as a Differential Diagnosis of Solid Retroperitoneal Masses: A Case Series and Review of the Literature. ⁶²	Levidou G...Manava P	35663681	2022
43	Differential transcriptomic profiling in ibrutinib-naïve versus ibrutinib-resistant Richter syndrome. ⁶²	Wang H...Ding W	34806797	2022
44	The complex karyotype landscape in chronic lymphocytic leukemia allows the refinement of the risk of Richter syndrome transformation. ⁶²	Visentin A...Trentin L	34092056	2022
45	Nodal hodgkin richter transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma. ⁶²	Behera G...Mishra P	35435414	2022
46	Novel Approaches for the Treatment of Patients with Richter's Syndrome. ⁶²	Iannello A...Vaisitti T	35294723	2022
47	Clinical outcome of allogeneic stem cell transplantation in patients with B-cell lymphoid malignancies following treatment with targeted small molecule inhibitors. ⁶²	Mukherjee A...Khouri IF	35225133	2022
48	Cellular Therapy Advances in Chronic Lymphocytic Leukemia and Richter's Syndrome. ⁶²	Bajwa A...Kittai AS	34991902	2022
49	Venetoclax plus dose-adjusted R-EPOCH for Richter syndrome. ⁶²	Davids MS...Brown JR	34788401	2022
50	Richter Transformation Arising From Chronic Lymphocytic Leukemia. ⁶²	Barnes Rogers B	35073287	2022

Sources

Genes for Richter's Syndrome

Genes related to Richter's Syndrome (0 elite genes):

(show all 20)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence
1	IGHV4-38-2	Immunoglobulin Heavy Variable 4-38-2	Protein Coding	7.64	DISEASES inferred ¹⁴
2	CD5	CD5 Molecule	Protein Coding	7.21	DISEASES inferred ¹⁴
3	ZAP70	Zeta Chain Of T Cell Receptor Associated Protein Kinase 70	Protein Coding	6.93	DISEASES inferred ¹⁴
4	BTK	Bruton Tyrosine Kinase	Protein Coding	6.62	DISEASES inferred ¹⁴
5	CD38	CD38 Molecule	Protein Coding	6.59	DISEASES inferred ¹⁴
6	NOTCH1	Notch Receptor 1	Protein Coding	6.48	DISEASES inferred ¹⁴
7	CD19	CD19 Molecule	Protein Coding	6.47	DISEASES inferred ¹⁴
8	TP53	Tumor Protein P53	Protein Coding	6.38	DISEASES inferred ¹⁴
9	BIRC3	Baculoviral IAP Repeat Containing 3	Protein Coding	6.06	DISEASES inferred ¹⁴
10	TCL1B	TCL1 Family AKT Coactivator B	Protein Coding	5.99	DISEASES inferred ¹⁴
11	BCL2	BCL2 Apoptosis Regulator	Protein Coding	5.97	DISEASES inferred ¹⁴
12	TCL1A	TCL1 Family AKT Coactivator A	Protein Coding	5.93	DISEASES inferred ¹⁴
13	CD79B	CD79b Molecule	Protein Coding	5.74	DISEASES inferred ¹⁴
14	MYC	MYC Proto-Oncogene, BHLH Transcription Factor	Protein Coding	5.72	DISEASES inferred ¹⁴
15	ATM	ATM Serine/Threonine Kinase	Protein Coding	5.68	DISEASES inferred ¹⁴
16	BCL6	BCL6 Transcription Repressor	Protein Coding	5.56	DISEASES inferred ¹⁴
17	RPS15	Ribosomal Protein S15	Protein Coding	5.54	DISEASES inferred ¹⁴
18	PAX5	Paired Box 5	Protein Coding	5.53	DISEASES inferred ¹⁴
19	CD22	CD22 Molecule	Protein Coding	5.45	DISEASES inferred ¹⁴
20	TNFRSF8	TNF Receptor Superfamily Member 8	Protein Coding	5.42	DISEASES inferred ¹⁴

Sources

Variations for Richter's Syndrome

Sources

Expression for Richter's Syndrome

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

Sources

Pathways for Richter's Syndrome

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

(show all 29)

#	Super pathways	Score	Top Affiliating Genes
1	Innate Immune System ⁶⁶ Show member pathways Immune System ⁶⁶ Neutrophil degranulation ⁶⁶	13.88	ZAP70 TP53 TNFRSF8 MYC CD79B CD22
2	ERK Signaling ⁶⁴ Show member pathways ILK Signaling ⁶⁴ MAPK Signaling ⁶⁴ Molecular Mechanisms of Cancer ⁶⁴ Rho Family GTPases ⁶⁴	13.74	ZAP70 TP53 TNFRSF8 NOTCH1 MYC BTK
3	PI3K-Akt signaling pathway ⁷⁴ Show member pathways Focal adhesion: PI3K-Akt-mTOR-signaling pathway ⁷⁴	12.85	TP53 TCL1B TCL1A MYC CD19 BCL2
4	Breast cancer pathway ⁷⁴ Show member pathways Embryonic stem cell pluripotency pathways ⁷⁴	12.37	TP53 NOTCH1 MYC ATM
5	NF-kappaB Signaling ³	12.32	ZAP70 TNFRSF8 PAX5 CD38 CD19 BTK
6	Immune response Fc epsilon RI pathway ²² Show member pathways Fc-epsilon receptor I signaling in mast cells ⁶¹ Immune response BCR pathway ²²	12.24	CD79B CD22 CD19 BTK BCL6
7	B Cell Receptor Signaling Pathway ⁶⁷ Show member pathways T Cell Receptor Signaling Pathway ⁶⁷	12.19	ZAP70 CD22 CD19 BTK BCL6
8	Integrated breast cancer pathway ⁷⁴	12.04	TP53 MYC BCL2 ATM
9	Interleukin-4 and Interleukin-13 signaling ⁶⁶	11.88	TP53 MYC BCL6 BCL2
10	TP53 Regulates Transcription of Cell Death Genes ⁶⁶ Show member pathways The IPAF inflammasome ⁶⁶ TP53 Regulates Transcription of Caspase Activators and Caspases ⁶⁶ TP53 Regulates Transcription of Death Receptors and Ligands ⁶⁶ TP53 Regulates Transcription of Genes Involved in Cytochrome C Release ⁶⁶ TP53 regulates transcription of several additional cell death genes whose specific roles in p53-dependent apoptosis remain uncertain ⁶⁶	11.84	TP53 BCL6 ATM
11	Notch Signaling Pathways ⁶⁵	11.84	BCL2 BCL6 MYC NOTCH1
12	Small cell lung cancer ⁷⁴	11.81	TP53 MYC BIRC3 BCL2
13	DNA damage response (only ATM dependent) ⁷⁴	11.73	TP53 MYC BCL6 BCL2 ATM
14	DNA IR-damage and cellular response via ATR ⁷⁴	11.72	TP53 BCL6 ATM
15	C-MYB transcription factor network ⁶¹	11.7	PAX5 MYC BIRC3 BCL2
16	Apoptosis and survival Anti-apoptotic TNFs/NF-kB/Bcl-2 pathway ²² Show member pathways Apoptosis and survival Anti-apoptotic TNFs/NF-kB/IAP pathway ²²	11.66	TNFRSF8 BIRC3 BCL2
17	miRNAs involved in DNA damage response ⁷⁴	11.62	TP53 MYC ATM
18	Extrafollicular B cell activation by SARS-CoV-2 ⁷⁴	11.61	PAX5 CD22 CD19 BCL6
19	Pathways affected in adenoid cystic carcinoma ⁷⁴	11.57	TP53 NOTCH1 ATM
20	Hepatitis C and hepatocellular carcinoma ⁷⁴	11.51	BIRC3 MYC TP53
21	DNA damage_ATM/ATR regulation of G1/S checkpoint ²²	11.4	TP53 MYC ATM
22	Antigen activates B Cell Receptor (BCR) leading to generation of second messengers ⁶⁶ Show member pathways B-Cell Receptor Complex ⁶⁴ CD22 mediated BCR regulation ⁶⁶	11.32	CD79B CD22 CD19 BTK
23	Integrated cancer pathway ⁷⁴	11.28	ATM BCL2 MYC TP53
24	BCR signaling pathway ⁶¹	11.17	CD79B CD22 CD19 BTK BCL6
25	Hematopoietic Stem Cells and Lineage-specific Markers ⁶⁵	11.16	NOTCH1 CD79B CD5 CD38 CD22 CD19
26	TP53 network ⁷⁴ Show member pathways Activation, translocation and oligomerization of BAX ⁶⁶	10.76	TP53 MYC BCL2 ATM
27	Mammary gland development pathway - Involution (Stage 4 of 4) ⁷⁴	10.63	TP53 MYC
28	PLK3 signaling events ⁶¹	10.38	TP53 ATM
29	MicroRNA network associated with chronic lymphocytic leukemia ⁷⁴	10.32	ZAP70 TP53 BCL2

Sources

GO Terms for Richter's Syndrome

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

#	Name	GO ID	Score	Top Affiliating Genes
1	protein-containing complex	GO:0032991	9.47	TP53 TCL1B TCL1A MYC CD19 BIRC3

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

(show all 15)

#	Name	GO ID	Score	Top Affiliating Genes
1	negative regulation of apoptotic process	GO:0043066	10.07	TP53 TCL1A MYC CD38 BIRC3 BCL6
2	cellular response to hypoxia	GO:0071456	10.06	TP53 NOTCH1 MYC BCL2
3	B cell proliferation	GO:0042100	10.02	CD38 BCL6 BCL2
4	immune system process	GO:0002376	9.95	ATM BCL6 BTK CD19 CD79B ZAP70
5	B cell activation	GO:0042113	9.91	ZAP70 CD22 BTK
6	positive regulation of apoptotic process	GO:0043065	9.9	ATM BCL2 BCL6 NOTCH1 TNFRSF8 TP53
7	response to gamma radiation	GO:0010332	9.88	TP53 MYC BCL2
8	B cell differentiation	GO:0030183	9.85	BCL2 BCL6 CD79B PAX5
9	mesenchymal cell development	GO:0014031	9.84	NOTCH1 BCL2
10	B cell lineage commitment	GO:0002326	9.8	TP53 BCL2
11	T cell lineage commitment	GO:0002360	9.69	TP53 BCL2
12	positive regulation of B cell proliferation	GO:0030890	9.56	CD38 BTK BCL6 BCL2
13	regulation of programmed cell death	GO:0043067	9.52	BIRC3 BCL2
14	negative regulation of leukocyte proliferation	GO:0070664	9.5	BTK BCL6
15	B cell receptor signaling pathway	GO:0050853	9.36	IGHV4-38-2 CD79B CD38 CD19 BTK BCL2