MCID: RCH001
MIFTS: 35

Richter's Syndrome malady

Rare diseases, Cancer diseases categories
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Summaries for Richter's Syndrome

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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 richter syndrome, is related to b-cell lymphomas and follicular lymphoma. An important gene associated with Richter's Syndrome is CD5 (CD5 molecule). Affiliated tissues include lymph node, liver and spleen, and related mouse phenotype immune system.

Aliases & Classifications for Richter's Syndrome

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Sources:
8Disease Ontology, 43NIH Rare Diseases, 62UMLS, 40NCIt, 58SNOMED-CT
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases, Cancer diseases


Aliases & Descriptions:

richter's syndrome 8 62
richter syndrome 8 43
richter transformation 43


External Ids:

Disease Ontology8 DOID:1703
SNOMED-CT58 277550009
NCIt40 C35424

Related Diseases for Richter's Syndrome

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Sources:
17GeneCards, 18GeneDecks
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Graphical network of diseases related to Richter's Syndrome:



Diseases related to richter's syndrome

Symptoms for Richter's Syndrome

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Drugs & Therapeutics for Richter's Syndrome

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Sources:
42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

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Genetic Tests for Richter's Syndrome

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Anatomical Context for Richter's Syndrome

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

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

Animal Models for Richter's Syndrome or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Richter's Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053878.7CD5, FOXN1, IRF4, MMP10

Publications for Richter's Syndrome

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Sources:
52PubMed
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Articles related to Richter's Syndrome:

(show top 50)    (show all 123)
idTitleAuthorsYear
1
Richter's syndrome: Utility of A^a8,F-FDG PET/CT. (23044071)
2013
2
Richter's syndrome with aberrant CD2 and CD4 expression. (22649134)
2012
3
Autologous and allogeneic stem-cell transplantation for transformed chronic lymphocytic leukemia (Richter's syndrome): A retrospective analysis from the chronic lymphocytic leukemia subcommittee of the chronic leukemia working party and lymphoma working party of the European Group for Blood and Marrow Transplantation. (22547610)
2012
4
Intrathecal liposomal cytarabine for treatment of leptomeningeal involvement in transformed (Richter's syndrome) and non-transformed B-cell chronic lymphocytic leukaemia in Spain: a report of seven cases. (20487439)
2010
5
Hodgkin's lymphoma variant of Richter's syndrome. (20437646)
2010
6
Haemophagocytic syndrome and Hodgkin's disease variant of Richter's syndrome after fludarabine for CLL. (20331737)
2010
7
Cutaneous Richter's syndrome, prognosis, and clinical, histological and immunohistological patterns: report of four cases and review of the literature. (20145381)
2010
8
Genomic profiling of Richter's syndrome: recurrent lesions and differences with de novo diffuse large B-cell lymphomas. (20014148)
2010
9
Richter's syndrome developing in a patient with adult onset Still's disease. (18607590)
2009
10
Recent advances in the diagnosis and therapy of Richter's syndrome. (17673808)
2007
11
EBV negative Richter's syndrome from a coexistent clone after salvage treatment with alemtuzumab in a CLL patient. (16838338)
2006
12
Clinical outcomes and prognostic factors in patients with Richter's syndrome treated with chemotherapy or chemoimmunotherapy with or without stem-cell transplantation. (16710033)
2006
13
Richter's syndrome--the downside of fludarabine? (16111529)
2005
14
Richter's syndrome: biology and therapy. (16053658)
2005
15
Cat scratch disease mimicking Richter's Syndrome in a patient with chronic lymphocytic leukemia. (15621836)
2005
16
Hodgkin's disease variant of Richter's syndrome in chronic lymphocytic leukaemia patients previously treated with fludarabine. (15813847)
2005
17
Distinct clonal origin in two cases of Hodgkin's lymphoma variant of Richter's syndrome associated With EBV infection. (15105659)
2004
18
Richter's syndrome presenting as a nasal lymphoma. (15223655)
2004
19
Hodgkin's type of Richter's syndrome in familial chronic lymphocytic leukemia treated with cladribine and cyclophosphamide. (12802926)
2003
20
Uncommon syndromes and treatment manifestations of malignancy: Case 3. Richter's syndrome heralded by refractory hypercalcemia. (12506188)
2003
21
Hodgkin's disease variant of Richter's syndrome: complete remission of the both malignancies after 14 years. (12911940)
2003
22
Phase II study of fludarabine, cytarabine (Ara-C), cyclophosphamide, cisplatin and GM-CSF (FACPGM) in patients with Richter's syndrome or refractory lymphoproliferative disorders. (12153163)
2002
23
Fractionated cyclophosphamide, vincristine, liposomal daunorubicin (daunoXome), and dexamethasone (hyperCVXD) regimen in Richter's syndrome. (11699397)
2001
24
Allogeneic haematopoietic transplantation for Richter's syndrome. (11054078)
2000
25
Hodgkin's disease variant of Richter's syndrome. Report of a case with diagnosis by fine needle biopsy. (9167708)
1997
26
Transformation of chronic lymphocytic leukemia to immunoblastic lymphoma (Richter's syndrome) (9180919)
1997
27
Hypercalcemia in a patient with chronic lymphocytic leukemia evolving into Richter's syndrome. (9172822)
1996
28
De novo CD5-positive and Richter's syndrome-associated diffuse large B cell lymphomas are genotypically distinct. (7541611)
1995
29
Richter's syndrome: report of a case. (8527977)
1995
30
Richter's syndrome showing pronounced lymphadenopathy in response to administration of granulocyte colony-stimulating factor. (7520793)
1994
31
Richter's syndrome associated with loss of response to transforming growth factor-beta. (8107492)
1994
32
Common clonal origin of chronic lymphocytic leukemia and high-grade lymphoma of Richter's syndrome. (7693038)
1993
33
Use of a retinoblastoma gene probe to investigate clonality in Richter's syndrome. (8231258)
1993
34
Genotypic analyses of Richter's syndrome. (1991269)
1991
35
The clonal origin of two cell populations in Richter's syndrome. (2361698)
1990
36
Hodgkin's disease variant of Richter's syndrome. Report of eight cases. (1689938)
1990
37
Single clonal origin of neoplastic B-cells with different immunoglobulin light chains in a patient with Richter's syndrome. (2112977)
1990
38
T cell malignancy in Richter's syndrome presenting as hyper IgM. Induction and characterization of a novel CD3+, CD4-, CD8+ T cell subset from phytohemagglutinin-stimulated patient's CD3+, CD4+, CD8+ leukemic T cells. (2523311)
1989
39
Primary central nervous system lymphoma as a variant of Richter's syndrome in two patients with chronic lymphocytic leukemia. (2766225)
1989
40
Richter's syndrome with identical immunoglobulin gene rearrangements in the chronic lymphocytic leukemia and the supervening non-Hodgkin lymphoma. (2811481)
1989
41
Intestinal lymphoma in a patient with chronic lymphocytic leukemia of atypical phenotype: Richter's syndrome of unusual presentation. (3138873)
1988
42
Large cell transformation of subclinical small lymphocytic leukemia/lymphoma: a variant of Richter's syndrome. (3498675)
1987
43
A case of Richter's syndrome due to the spontaneous proliferation of two immunologically unrelated B cell malignancies. (3108099)
1987
44
Reed-Sternberg-like cells in Richter's syndrome express granulocytic-associated-antigen (Leu-M1) (3706214)
1986
45
Gastrointestinal Richter's syndrome. (3770738)
1986
46
Philadelphia (Ph), 14q+ and 1q+ chromosomes in immunoblastic phase (Richter's syndrome) in a patient with T chronic lymphocytic leukaemia. (3093326)
1986
47
Richter's syndrome mimicking chronic colitis. A patient with diffuse histiocytic lymphoma complicating chronic lymphocytic leukemia. (3943435)
1986
48
Case 31-1983: Richter's syndrome. (6606779)
1983
49
Richter's syndrome: a blastic transformation of chronic lymphocyte leukemia. (7335044)
1981
50
Richter's syndrome: diffuse histiocytic lymphoma in patients with chronic lymphocytic leukemia. A report of five cases and review of the literature. (6989238)
1980

Variations for Richter's Syndrome

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Expression for genes affiliated with Richter's Syndrome

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Sources:
2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Richter's Syndrome

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Pathways for genes affiliated with Richter's Syndrome

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Compounds for genes affiliated with Richter's Syndrome

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GO Terms for genes affiliated with Richter's Syndrome

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16Gene Ontology
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Cellular components related to Richter's Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.6IL19, MMP10, CPA4

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

idNameGO IDScoreTop Affiliating Genes
1transcription factor bindingGO:0081349.6FOXN1, IRF4
2sequence-specific DNA bindingGO:0435658.7GTF2E1, FOXN1, IRF4

Products for genes affiliated with Richter's Syndrome

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Sources for Richter's Syndrome

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet