MCID: RCH001
MIFTS: 46

Richter's Syndrome malady

Immune category

Summaries for Richter's Syndrome

Sources:
43NIH Rare Diseases, 33MalaCards
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NIH Rare Diseases:43 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: Richter's Syndrome, also known as large cell lymphoma which develops after treatment for chronic lymphocytic leukemia, is related to t-cell leukemia and follicular lymphoma. An important gene associated with Richter's Syndrome is CD5 (CD5 molecule), and among its related pathways are Hematopoietic cell lineage and Chronic myeloid leukemia. The compounds butyrate and p00048 have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and spinal cord, and related mouse phenotypes are tumorigenesis and liver/biliary system.

Aliases & Classifications for Richter's Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 61UMLS, 40NCIt, 57SNOMED-CT
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Immune


Aliases & Descriptions:

richter's syndrome 8 61
large cell lymphoma which develops after treatment for chronic lymphocytic leukemia 43
richter syndrome 43


External Ids:

Disease Ontology8 DOID:1703
SNOMED-CT57 277550009
NCIt40 C35424

Related Diseases for Richter's Syndrome

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Richter's Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 90)
idRelated DiseaseScoreTop Affiliating Genes
1t-cell leukemia30.4PTHLH, NOTCH1, ATM, IRF4
2follicular lymphoma30.0CSF2, IRF4, CD5, BCR
3b-cell chronic lymphocytic leukemia30.0ATM, CD5, CD38, BCR
4kotzot-richter syndrome10.4
5lymphoma, diffuse large cell10.4
6leukemia, t-cell, chronic10.3
7n syndrome10.2
8leukemia/lymphoma, t-cell10.2
9graft versus host disease10.0
10colon lymphoma10.0
11mantle cell lymphoma10.0
12char syndrome10.0
13young syndrome10.0
14leukemia, b-cell, chronic10.0
15chronic graft versus host disease10.0
16lymphoplasmacytoid lymphoma10.0
17leukemia, chronic lymphocytic 210.0
18hematopoietic stem cell transplantation10.0
19severe combined immunodeficiency10.0CD38
20hepatitis c10.0BCR
21adult t-cell leukemia10.0PTHLH
22systemic lupus erythematosus10.0CRP
23plasmacytoma10.0IRF4
24rhabdoid tumor10.0BCR
25fanconi's anemia10.0ATM
26kidney cancer10.0CSF2
27adenocarcinoma in situ10.0CDKN2A
28hypersensitivity reaction type ii disease10.0CD5
29pituitary adenoma10.0IFI27
30respiratory failure10.0CRP
31smooth muscle tumor10.0IFI27
32lymphoma, malt, somatic10.0CD5, IRF4
33laryngeal carcinoma10.0IFI27
34pancreatitis10.0CRP
35arthritis10.0CSF2, CRP
36leukemoid reaction10.0CSF2
37thymoma10.0CD5, IFI27
38thyroid cancer10.0CDKN1A
39multiple myeloma10.0IRF4, CDKN2A, CD38
40tongue cancer10.0CDKN1A
41gastrointestinal stromal tumor10.0BCR, IFI27
42prolymphocytic leukemia10.0ATM, CD5, CD38
43esophageal cancer10.0CDKN2A
44deficiency anemia10.0CSF2, CRP, CD5
45myelodysplastic syndromes10.0SPN, CSF2
46chondrosarcoma10.0PTHLH
47li-fraumeni syndrome10.0ATM, CDKN2A, CDKN1A
48rheumatoid arthritis10.0CSF2, CRP, CD5
49thrombocytopenia10.0SPN, CSF2
50clear cell adenocarcinoma10.0PTHLH, IFI27

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



Diseases related to richter's syndrome

Clinical Features for Richter's Syndrome

Drugs & Therapeutics for Richter's Syndrome

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Richter's Syndrome

Drug clinical trials:

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Search NIH Clinical Center for Richter's Syndrome

Search CenterWatch for Richter's Syndrome

Genetic Tests for Richter's Syndrome

Anatomical Context for Richter's Syndrome

Sources:
33MalaCards
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MalaCards organs/tissues related to Richter's Syndrome:

33
Skin, Liver, Spinal cord, Brain, Lymph node, Spleen, Bone marrow, Small intestine, Colon, Monocytes, T cells, B cells

Animal Models for Richter's Syndrome or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Richter's Syndrome

Sources:
51PubMed
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Articles related to Richter's Syndrome:

(show top 50)    (show all 150)
idTitleAuthorsYear
1
Richter syndrome: chronic lymphocytic leukemia transformation into Hodgkin's disease. (24266095)
2013
2
Richter's syndrome: Utility of A^a8,F-FDG PET/CT. (23044071)
2013
3
An unusual presentation of a chronic lymphocytic leukemia patient with 17p deletion after reduced-intensity transplantation: Richter syndrome and concomitant graft-versus-host disease--case report. (23747187)
2013
4
Two main genetic pathways lead to the transformation of chronic lymphocytic leukemia to Richter syndrome. (24004666)
2013
5
Clinical and economic burden of Richter syndrome in inpatient cases of chronic lymphocytic leukemia within the United States, 2001-2010. (23772638)
2013
6
The impact of initial fludarabine therapy on transformation to Richter syndrome or prolymphocytic leukemia in patients with chronic lymphocytic leukemia: analysis of an intergroup trial (CALGB 9011). (22897733)
2013
7
Haemophagocytic syndrome and Hodgkin's disease variant of Richter's syndrome after fludarabine for CLL. (20331737)
2010
8
Stereotyped B-cell receptor is an independent risk factor of chronic lymphocytic leukemia transformation to Richter syndrome. (19509140)
2009
9
Richter's syndrome developing in a patient with adult onset Still's disease. (18607590)
2009
10
Clonality analysis and mutation status of IgVH genes in classic Richter's syndrome]. (19031714)
2008
11
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
12
CD38 gene polymorphism and chronic lymphocytic leukemia: a role in transformation to Richter syndrome? (18424664)
2008
13
Primary effusion lymphoma presenting as Richter's syndrome. (17573910)
2007
14
EBV negative Richter's syndrome from a coexistent clone after salvage treatment with alemtuzumab in a CLL patient. (16838338)
2006
15
Clinical outcomes and prognostic factors in patients with Richter's syndrome treated with chemotherapy or chemoimmunotherapy with or without stem-cell transplantation. (16710033)
2006
16
Hodgkin's disease variant of Richter's syndrome clonally related to chronic lymphocytic leukemia arises in ZAP-70 negative mutated CLL. (16223567)
2006
17
Richter's syndrome--the downside of fludarabine? (16111529)
2005
18
Richter's syndrome: biology and therapy. (16053658)
2005
19
Hodgkin's disease variant of Richter's syndrome in chronic lymphocytic leukaemia patients previously treated with fludarabine. (15813847)
2005
20
Richter syndrome: biology, incidence, and therapeutic strategies. (15578683)
2005
21
Relationship between the mutational status of VH genes and pathogenesis of diffuse large B-cell lymphoma in Richter's syndrome. (14671632)
2004
22
Second malignancies and Richter's syndrome in patients with chronic lymphocytic leukemia. (15763979)
2004
23
Richter's syndrome in the brain first manifested as an ischaemic stroke. (15360010)
2004
24
Second malignancies and Richter's syndrome in patients with chronic lymphocytic leukaemia treated with cladribine. (14746857)
2004
25
Hodgkin's type of Richter's syndrome in familial chronic lymphocytic leukemia treated with cladribine and cyclophosphamide. (12802926)
2003
26
Fractionated cyclophosphamide, vincristine, liposomal daunorubicin, and dexamethasone plus rituximab and granulocyte-macrophage-colony stimulating factor (GM-CSF) alternating with methotrexate and cytarabine plus rituximab and GM-CSF in patients with Richter syndrome or fludarabine-refractory chronic lymphocytic leukemia. (12655528)
2003
27
Uncommon syndromes and treatment manifestations of malignancy: Case 3. Richter's syndrome heralded by refractory hypercalcemia. (12506188)
2003
28
Richter's syndrome after allogeneic stem cell transplantation for chronic lymphocytic leukaemia successfully treated by withdrawal of immunosuppression, and donor lymphocyte infusion. (12621484)
2003
29
Richter syndrome in B-cell chronic lymphocytic leukemia. (12675762)
2003
30
Genetic imbalances in progressed B-cell chronic lymphocytic leukemia and transformed large-cell lymphoma (Richter's syndrome). (12213724)
2002
31
Richter's syndrome following cladribine therapy for chronic lymphocytic leukemia first manifested as pathologic fracture of the femur. (11697509)
2001
32
Epstein-Barr virus associated Richter's syndrome accompanied by interstitial pneumonia]. (10390889)
1999
33
Hodgkin's disease type Richter's syndrome in chronic lymphocytic leukemia. (10374892)
1999
34
Richter's syndrome in a case of atypical chronic lymphocytic leukaemia with the t(11;14)(q13;q32): role for a p53 exon 7 gene mutation. (8603003)
1996
35
Different clonal origin of B-cell populations of chronic lymphocytic leukemia and large-cell lymphoma in Richter's syndrome. (7486574)
1995
36
Frequent somatic mutations in D and/or JH segments of Ig gene in WaldenstrAPm's macroglobulinemia and chronic lymphocytic leukemia (CLL) with Richter's syndrome but not in common CLL. (7703494)
1995
37
Richter's syndrome associated with loss of response to transforming growth factor-beta. (8107492)
1994
38
Molecular genetic demonstration of the diverse evolution of Richter's syndrome (chronic lymphocytic leukemia and subsequent large cell lymphoma). (8118038)
1994
39
A cultured malignant B-1 line serves as a model for Richter's syndrome. (8046247)
1994
40
Common clonal origin of chronic lymphocytic leukemia and high-grade lymphoma of Richter's syndrome. (7693038)
1993
41
Use of a retinoblastoma gene probe to investigate clonality in Richter's syndrome. (8231258)
1993
42
Discordant differentiation antigen pattern in a case of Richter's syndrome with monoclonal idiotype expression and immunoglobulin gene rearrangement. (2386740)
1990
43
Focal Candida hepatitis in a patient with Richter's syndrome. (2234526)
1990
44
Immunophenotypic and gene probe analysis of a case of Richter's syndrome. (2644809)
1989
45
Large cell transformation of subclinical small lymphocytic leukemia/lymphoma: a variant of Richter's syndrome. (3498675)
1987
46
A case of Richter's syndrome due to the spontaneous proliferation of two immunologically unrelated B cell malignancies. (3108099)
1987
47
J-chain-producing immunoblastic lymphoma in a case of Richter's syndrome. Immunohistochemical evidence for a gradual malignant transformation of a single B-cell clone and flow cytophotometric data. (6812283)
1982
48
Richter's syndrome: diffuse histiocytic lymphoma in patients with chronic lymphocytic leukemia. A report of five cases and review of the literature. (6989238)
1980
49
Apparent clinical remission in chronic lymphocytic leukemia with terminal features of Richter's syndrome. (6778786)
1980
50
Richter's syndrome. A terminal complication of chronic lymphocytic leukemia with distinct clinicopathologic features. (1096589)
1975

Genetic Variations for Richter's Syndrome

Expression for genes affiliated with Richter's Syndrome

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Richter's Syndrome

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

Pathways for genes affiliated with Richter's Syndrome

Sources:
30KEGG, 38NCBI BioSystems Database, 12EMD Millipore, 52QIAGEN, 54Reactome
See all sources

Pathways related to Richter's Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.2CD38, CD5, CSF2
2
Hide members
10.2CDKN1A, CDKN2A, BCR
3
Hide members
10.2CDKN2A, ATM, CDKN1A
410.2CDKN1A, CDKN2A, ATM
5
Transcription P53 signaling pathway
Hide members
10.2CDKN1A, CDKN2A, ATM
6
Hide members
10.2ATM, CDKN2A, CDKN1A
710.1ATM, CDKN1A, CDKN2A, NOTCH1
810.1CSF2, ATM, CDKN2A, CDKN1A

Compounds for genes affiliated with Richter's Syndrome

Sources:
45Novoseek, 60Tocris Bioscience, 50PharmGKB, 29IUPHAR, 11DrugBank, 24HMDB, 2BitterDB
See all sources

Compounds related to Richter's Syndrome according to GeneCards/GeneDecks:

(show all 50)
idCompoundScoreTop Affiliating Genes
1butyrate4510.7CSF2
2p000484510.7CDKN1A, IFI27
3optimax4510.7CDKN1A, IFI27
4eb 108945 6011.6IFI27, CDKN1A, PTHLH
5acnu4510.6CDKN1A, CDKN2A
6tamoxifen45 50 29 1113.6PTHLH, CDKN1A
7cytochalasin b4510.6CSF2, SPN, BCR
8rituximab45 50 1112.6BCR, CD38, CRP, CSF2
9neopterin4510.5CSF2, CRP, CD38
10roscovitine4510.5CDKN1A, CDKN2A, IFI27
11mevastatin45 29 1112.5CDKN1A, IFI27
12hydroxyurea45 50 1112.5CDKN1A, CDKN2A, ATM, CSF2
13serine4510.5BCR
14bleomycin45 1111.5CSF2, ATM, PTHLH
15lovastatin45 50 60 29 1114.5PTHLH, CDKN1A, CDKN2A, IFI27
1615-deoxy-delta-12,14-prostaglandin j24510.5CDKN1A, CSF2, IFI27
17cyclophosphamide45 50 1112.5CSF2, CRP, PTHLH, BCR
18rosiglitazone45 50 29 11 2414.5PTHLH, CDKN1A, CRP, IFI27
19dexamethasone45 50 29 1113.5NOTCH1, CD38, CDKN2A, IFI27
20calcitriol45 60 11 2413.4IFI27, CSF2, CD38, CDKN1A, PTHLH
21agar4510.4PTHLH, CDKN1A, CDKN2A, CSF2
22actinomycin d4510.4CSF2, CDKN2A, CD38, CDKN1A, PTHLH
23n acetylcysteine4510.4IFI27, CRP, ATM, CDKN2A, CDKN1A
24rapamycin4510.4IFI27, CRP, ATM, CDKN1A, NOTCH1
25paclitaxel45 50 1112.4CDKN1A, CDKN2A, IFI27
26adpribose4510.4IFI27, CD38, CDKN1A
27cytarabine45 50 1112.4BCR, IFI27, CSF2, ATM, CDKN2A, CDKN1A
28azithromycin45 1111.4CSF2, CRP
29thymidine45 2411.3IFI27, CSF2, CDKN1A, PTHLH, BCR
30azathioprine45 50 2 1113.3PTHLH, CDKN2A, CRP
31ly2940024510.3IFI27, CRP, ATM, CDKN1A, NOTCH1, BCR
32lactate4510.3IFI27, CSF2, CRP, ATM, PTHLH
33doxorubicin45 50 1112.3IFI27, CSF2, ATM, CDKN2A, CDKN1A, NOTCH1
34cisplatin45 50 60 1113.3IFI27, CSF2, ATM, CDKN2A, CDKN1A, PTHLH
35vitamin d4510.3IFI27, CSF2, CDKN1A, PTHLH
36hpvs4510.3CDKN2A, CDKN1A
37phosphatidylinositol4510.3IFI27, CSF2, ATM, CD38, NOTCH1, PTHLH
38genistein45 29 60 2 11 2415.3IFI27, CSF2, ATM, CDKN2A, CDKN1A, NOTCH1
39paraffin4510.3IFI27, ATM, CD5, CDKN2A, CDKN1A, PTHLH
40h2o24510.2CSF2, CDKN2A, CDKN1A, SPN, BCR
41vegf4510.2IFI27, CSF2, CRP, CDKN2A, CDKN1A, NOTCH1
42oxygen45 2411.2IFI27, ATM, CDKN2A, CDKN1A, NOTCH1, PTHLH
43thymidylate4510.2CDKN1A, CDKN2A, IFI27
44retinoic acid45 2411.2IFI27, CSF2, CDKN2A, CD38, CDKN1A, NOTCH1
45threonine4510.2IFI27, ATM, CDKN2A, CDKN1A, NOTCH1, PTHLH
46lipid4510.1IFI27, CRP, ATM, CDKN2A, CD38, NOTCH1
47vincristine45 50 1112.0PTHLH, CSF2
48calcium45 50 11 2413.0IFI27, CRP, CD5, CDKN2A, CD38, NOTCH1
49pd 98,0594510.0IFI27, CSF2, CDKN1A, BCR
50tyrosine459.9IFI27, CSF2, ATM, LILRA4, CD5, CD38

GO Terms for genes affiliated with Richter's Syndrome

Sources:
16Gene Ontology
See all sources

Biological processes related to Richter's Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1somatic stem cell divisionGO:04810310.4NOTCH1, CDKN2A
2myeloid dendritic cell differentiationGO:04301110.3IRF4, CSF2
3replicative senescenceGO:09039910.3ATM, CDKN2A
4apoptotic signaling pathwayGO:09719010.2SPN, CD38, CD5, IFI27
5positive regulation of DNA damage response, signal transduction by p53 class mediatorGO:04351710.2CDKN2A, ATM
6negative regulation of B cell proliferationGO:03088910.0CDKN2A, ATM

Molecular functions related to Richter's Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cyclin-dependent protein serine/threonine kinase inhibitor activityGO:00486110.3CDKN1A, CDKN2A

Products for genes affiliated with Richter's Syndrome

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Richter's Syndrome

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet