MCID: RCH001
MIFTS: 29

Richter's Syndrome malady

Rare diseases category

Summaries for Richter's Syndrome

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NIH Rare Diseases:41 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 based summary: 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, spleen and liver, and related mouse phenotype immune system.

Aliases & Classifications for Richter's Syndrome

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Richter's Syndrome, Aliases & Descriptions:

Name: Richter's Syndrome 9 60
Richter Syndrome 9 41
 
Richter Transformation 41


Classifications:

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


External Ids:

Disease Ontology9 DOID:1703
SNOMED-CT55 277550009
NCIt38 C35424

Related Diseases for Richter's Syndrome

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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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Drug clinical trials:

Search ClinicalTrials for Richter's Syndrome

Search NIH Clinical Center for Richter's Syndrome

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:

31
Lymph node, Spleen, Liver, B cells, Bone, Brain, T cells, Bone marrow, Spinal cord, Colon, Skin, Monocytes

Animal Models for Richter's Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Richter's Syndrome:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053878.7CD5, FOXN1, IRF4, MMP10

Publications for Richter's Syndrome

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Articles related to Richter's Syndrome:

(show top 50)    (show all 128)
idTitleAuthorsYear
1
Ibrutinib has some activity in Richter's syndrome. (25635530)
2015
2
Single arm NCRI phase II study of CHOP in combination with Ofatumumab in induction and maintenance for patients with newly diagnosed Richter's syndrome. (25775024)
2015
3
Hemophagocytic Lymphohistiocytosis With Cerebral Involvement in Richter's Syndrome. (25534389)
2014
4
Richter's syndrome: Utility of A^a8,F-FDG PET/CT. (23044071)
2013
5
Richter's syndrome with aberrant CD2 and CD4 expression. (22649134)
2012
6
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
7
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
8
Hodgkin's lymphoma variant of Richter's syndrome. (20437646)
2010
9
Haemophagocytic syndrome and Hodgkin's disease variant of Richter's syndrome after fludarabine for CLL. (20331737)
2010
10
Cutaneous Richter's syndrome, prognosis, and clinical, histological and immunohistological patterns: report of four cases and review of the literature. (20145381)
2010
11
Genomic profiling of Richter's syndrome: recurrent lesions and differences with de novo diffuse large B-cell lymphomas. (20014148)
2010
12
Richter's syndrome developing in a patient with adult onset Still's disease. (18607590)
2009
13
Recent advances in the diagnosis and therapy of Richter's syndrome. (17673808)
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
Richter's syndrome--the downside of fludarabine? (16111529)
2005
17
Richter's syndrome: biology and therapy. (16053658)
2005
18
Cat scratch disease mimicking Richter's Syndrome in a patient with chronic lymphocytic leukemia. (15621836)
2005
19
Hodgkin's disease variant of Richter's syndrome in chronic lymphocytic leukaemia patients previously treated with fludarabine. (15813847)
2005
20
Distinct clonal origin in two cases of Hodgkin's lymphoma variant of Richter's syndrome associated With EBV infection. (15105659)
2004
21
Richter's syndrome presenting as a nasal lymphoma. (15223655)
2004
22
Hodgkin's type of Richter's syndrome in familial chronic lymphocytic leukemia treated with cladribine and cyclophosphamide. (12802926)
2003
23
Uncommon syndromes and treatment manifestations of malignancy: Case 3. Richter's syndrome heralded by refractory hypercalcemia. (12506188)
2003
24
Hodgkin's disease variant of Richter's syndrome: complete remission of the both malignancies after 14 years. (12911940)
2003
25
Fractionated cyclophosphamide, vincristine, liposomal daunorubicin (daunoXome), and dexamethasone (hyperCVXD) regimen in Richter's syndrome. (11699397)
2001
26
Allogeneic haematopoietic transplantation for Richter's syndrome. (11054078)
2000
27
Hodgkin's disease variant of Richter's syndrome. Report of a case with diagnosis by fine needle biopsy. (9167708)
1997
28
Transformation of chronic lymphocytic leukemia to immunoblastic lymphoma (Richter's syndrome) (9180919)
1997
29
Hypercalcemia in a patient with chronic lymphocytic leukemia evolving into Richter's syndrome. (9172822)
1996
30
De novo CD5-positive and Richter's syndrome-associated diffuse large B cell lymphomas are genotypically distinct. (7541611)
1995
31
Richter's syndrome: report of a case. (8527977)
1995
32
Richter's syndrome showing pronounced lymphadenopathy in response to administration of granulocyte colony-stimulating factor. (7520793)
1994
33
Richter's syndrome associated with loss of response to transforming growth factor-beta. (8107492)
1994
34
Common clonal origin of chronic lymphocytic leukemia and high-grade lymphoma of Richter's syndrome. (7693038)
1993
35
Use of a retinoblastoma gene probe to investigate clonality in Richter's syndrome. (8231258)
1993
36
Genotypic analyses of Richter's syndrome. (1991269)
1991
37
The clonal origin of two cell populations in Richter's syndrome. (2361698)
1990
38
Hodgkin's disease variant of Richter's syndrome. Report of eight cases. (1689938)
1990
39
Single clonal origin of neoplastic B-cells with different immunoglobulin light chains in a patient with Richter's syndrome. (2112977)
1990
40
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
41
Primary central nervous system lymphoma as a variant of Richter's syndrome in two patients with chronic lymphocytic leukemia. (2766225)
1989
42
Richter's syndrome with identical immunoglobulin gene rearrangements in the chronic lymphocytic leukemia and the supervening non-Hodgkin lymphoma. (2811481)
1989
43
Large cell transformation of subclinical small lymphocytic leukemia/lymphoma: a variant of Richter's syndrome. (3498675)
1987
44
A case of Richter's syndrome due to the spontaneous proliferation of two immunologically unrelated B cell malignancies. (3108099)
1987
45
Reed-Sternberg-like cells in Richter's syndrome express granulocytic-associated-antigen (Leu-M1) (3706214)
1986
46
Gastrointestinal Richter's syndrome. (3770738)
1986
47
Philadelphia (Ph), 14q+ and 1q+ chromosomes in immunoblastic phase (Richter's syndrome) in a patient with T chronic lymphocytic leukaemia. (3093326)
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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Search GEO for disease gene expression data for Richter's Syndrome.

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

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:00055768.6IL19, MMP10, CPA4

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

idNameGO IDScoreTop Affiliating Genes
1transcription factor bindingGO:00081349.6FOXN1, IRF4
2sequence-specific DNA bindingGO:00435658.7GTF2E1, FOXN1, IRF4

Products for genes affiliated with Richter's Syndrome

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Sources for Richter's Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet